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{{Bleeding diathesis}}
{{Bleeding diathesis}}
{{CMG}}; {{MJ}}
{{CMG}}; {{AE}} {{MJ}}, {{N.F}}, {{S.G.}}
 
{{SK}}  Hypocoagulopathy; blood coagulation disorders; hemorrhagic diathesis; hemostasis abnormality; bleeding tendency
 


== Overview ==
== Overview ==
Bleeding diathesis is susceptibility to bleed due to [[Coagulopathy|coagulopathy disorders]] or [[platelets]] disorders. These diseases can occur due to a disorder of [[homeostasis]], localized process ([[tissue]] injury), or [[Medication|medications]]. Bleeding diathesis can be resulted from [[vessel wall]] injury, [[platelet]] disorders, and [[coagulation factor]] disorders. Clinical manifestation of bleeding disorders can have a wide range of symptoms from asymptomatic to symptomatic massive and life threatening bleeding. [[Platelet]] disorders mostly have skin manifestations such as [[Petechia|petechiae]], and  [[ecchymoses]]. In order to find the cause of hypo-[[coagulopathy]]; there are established laboratory tests, such as [[peripheral blood smear]], [[platelet]] count and [[platelet]] function analysis, [[coagulation factor]] deficiencies and inhibitors, [[fibrinolysis]] tests (eg. [[D-dimer]] level), [[bleeding time|bleeding time (BT)]], [[prothrombin time|prothrombin time (PT)]], [[Partial thromboplastin time|activated partial thromboplastin time (aPTT)]], [[thrombin time]] (TT), and reptilase time. In the case of any abnormal bleeding, first line of screening tests are [[Complete blood count|CBC]], [[PT]], [[Partial thromboplastin time|PTT]], [[Bleeding time|BT]], and [[Thrombin time|TT]].


== Classification ==
== Classification ==
Disorders of hemostasis can be classified into two main categories: platelet disorders, and disorders of coagulation. Each category can be further classified as bellow:
{{Family tree/start}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | A01=Abnormal hemostasis }}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | }}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | B01 | | | | | | | | | | | | | | | | | |B01=<br>• Patient history-sign & symptom: deep soft tissue & mucocutaneus bleeding <br>• Screen test CBC-Plt-PT&PTT-BT-TT }}
{{familytree | | | | | | | | | | | | | | |,|-|-|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|-|-|-|-|-|.| | | | | | | | | }}
{{familytree | | | | | | | | | | | | | | B01 | | | | | | | | | | | | | | | | | | | | | B02 | | | | | | | | |B01=Hx of deep soft tissue bleeding |B02=Hx of mucocotaneus bleeding | | | }}
{{familytree | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | }}
{{familytree | | | | | | | | | | | | | | C01 | | | | | | | | | | | | | | | | | | | | | | C02 | | | | | | | | |C01=Coagulopathy |C02=Plt disorder | | | }}
{{familytree | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | |,|-|-|^|-|.| | | | | | | | }}
{{familytree | | | | | | | | | | | | | | D01 | | | | | | | | | | | | | | | | | | | D02 | | | D03 | | | | | | | |D01=Family history |D02=Normal plt count |D03=Low plt count | | | }}
{{familytree | | | | |,|-|-|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|-|-|-|-|.| | | | | | | | |!| | | | | | | | | | | }}
{{familytree | | | | | E01 | | | | | | | | | | | | | | | | | | | E02 | | | | | | | E03 | | | | | | |E01=(+)<br>• Inherited coagulpathy |E02=(-)<br>• Acquired coagulopathy |E03=Functional Plt disorder | | |}}
{{familytree | |,|-|-|-|+|-|-|-|.| | | | |,|-|-|-|v|-|-|-|v|-|-|-|+|-|-|-|-|-|-|.| |!| | | }}
{{familytree | F01 | | F02 | | F03 | | | F04 | | F05 | | F06 | | F07 | | | | | F08 |!| | | |F01=↑Plt <br>• Hemophillia <br>• VWD <br>• Factor VIII or IX deficiency |F02=↑PT <br>• Factor VII deficiency |F03=↑PT&↑PTT <br>• Fibrinogen deficiency <br>• Factor II deficiency <br>• Factor V deficiency <br>• Factor X deficiency |F04=↑PTT <br>• Factor inhibitor <br>• Anti phospholipid Ab syndrome |F05=↑PT <br>• Factor inhibitor <br>• Vit K deficiency <br>• Liver disease |F06=↑PT&↑PTT <br>• Factor inhibit <br>• DIC <br>• Liver failure <br>• Late stage of Vit K deficiency |F07=↑ Afibrinogenemia <br>• Heparin inhibitor <br>• Direct thrombin inhibitor |F08=Abnormal solobity <br>• Factor XIII deficiency <br>• Cross-linkin inhibitor | | |}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | |!| | | | | | | | | | | }}
{{familytree | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | |,|-|^|-|.| | | | | | | | | }}
{{familytree | | | | | | | | | | | | | | | | | | | | | | G01 | | | | | | | | | G02 | | G03 |G01=Not corrected with mixing with NL plasma |G02=HX(+) |G03=HX(-) | | | }}
{{familytree | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | |!| | | |!| | | | | |}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | H01 | | | | | | | | | H02 | | H03 | | |H01=<br>• Factor inhibitors <br>• Lupus  anti coagulant <br>• DIC <br>• Heparin or direct thrombin inhibitors |H02=Congenital |H03=Acquired | | | }} 
{{Family tree/end}}
== Differentiating Bleeding Disorders from Other Diseases ==
Different causes of bleeding disorders can be differentiated based on their clinical manifestation and laboratory findings.
These features have discussed in the below table:
{|
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory
! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! colspan="6" align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical manifestation
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory testing
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT
|-
! rowspan="16" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Platelet disorders
! rowspan="7" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombocytopenia]]
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Infection]]-Induced [[thrombocytopenia]]<ref name="HerbingerSchunk2012">{{cite journal|last1=Herbinger|first1=K.-H.|last2=Schunk|first2=M.|last3=Nothdurft|first3=H. D.|last4=von Sonnenburg|first4=F.|last5=Löscher|first5=T.|last6=Bretzel|first6=G.|title=Comparative study on infection-induced thrombocytopenia among returned travellers|journal=Infection|volume=40|issue=4|year=2012|pages=373–379|issn=0300-8126|doi=10.1007/s15010-012-0242-9}}</ref>


== Differential Diagnosis ==
| align="left" style="background:#F5F5F5;" |
{| class="wikitable"
* History of prior infection
|+
| align="center" style="background:#F5F5F5;" | +
!
| align="center" style="background:#F5F5F5;" | +
!
| align="center" style="background:#F5F5F5;" | +
!
| align="center" style="background:#F5F5F5;" | +
!
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | −
|-
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Medication]]-Induced [[Thrombocytopenia|Thrombocytopenia]]<ref name="EltingCantor2003">{{cite journal|last1=Elting|first1=Linda S.|last2=Cantor|first2=Scott B.|last3=Martin|first3=Charles G.|last4=Hamblin|first4=Lois|last5=Kurtin|first5=Danna|last6=Rivera|first6=Edgardo|last7=Vadhan-Raj|first7=Saroj|last8=Benjamin|first8=Robert S.|title=Cost of chemotherapy-induced thrombocytopenia among patients with lymphoma or solid tumors|journal=Cancer|volume=97|issue=6|year=2003|pages=1541–1550|issn=0008-543X|doi=10.1002/cncr.11195}}</ref>
| align="left" style="background:#F5F5F5;" |
*History of [[Medication|medications]] such as:
** [[Furosemide]]
** [[Non-steroidal anti-inflammatory drug|Nonsteroidal anti-inflammatory drugs]] ([[Non-steroidal anti-inflammatory drug|NSAIDs]])
** [[Penicillin]]
** [[Quinidine]]
** [[Quinine]]
** [[Ranitidine]]
** [[Sulfonamide (medicine)|Sulfonamides]]
** [[Linezolid]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Most important part of treatment is discontinuing of the medication.
|-
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Heparin-Induced Thrombocytopenia|Heparin-Induced thrombocytopenia]]<ref name="Miller2003">{{cite journal|last1=Miller|first1=Penny L.|title=Heparin-induced Thrombocytopenia Recognition and Treatment|journal=AORN Journal|volume=78|issue=1|year=2003|pages=79–89|issn=00012092|doi=10.1016/S0001-2092(06)61348-3}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Thrombosis]]
* Unexplained [[thrombocytopenia]] up to 3 weeks after the end of [[heparin]] therapy
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | For more information click here: [[Heparin-induced thrombocytopenia]].
|-
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Idiopathic thrombocytopenic purpura|Immune Thrombocytopenic Purpura]]<ref name="CurtisKaliszewski2006">{{cite journal|last1=Curtis|first1=Brian R.|last2=Kaliszewski|first2=James|last3=Marques|first3=Marisa B.|last4=Saif|first4=M. Wasif|last5=Nabelle|first5=Lisle|last6=Blank|first6=Jules|last7=McFarland|first7=Janice G.|last8=Aster|first8=Richard H.|title=Immune-mediated thrombocytopenia resulting from sensitivity to oxaliplatin|journal=American Journal of Hematology|volume=81|issue=3|year=2006|pages=199–201|issn=0361-8609|doi=10.1002/ajh.20516}}</ref>
| align="left" style="background:#F5F5F5;" |
* History of prior [[infection]] or no history
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | −
|-
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited [[Thrombocytopenia]]<ref name="Drachman2004">{{cite journal|last1=Drachman|first1=J. G.|title=Inherited thrombocytopenia: when a low platelet count does not mean ITP|journal=Blood|volume=103|issue=2|year=2004|pages=390–398|issn=0006-4971|doi=10.1182/blood-2003-05-1742}}</ref>
| align="left" style="background:#F5F5F5;" |
* Positive family history
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | −
|-
|-
| rowspan="8" |Platelet disorders
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombotic thrombocytopenic purpura|Thrombotic Thrombocytopenic Purpura]]<ref name="George2006">{{cite journal|last1=George|first1=James N.|title=Thrombotic Thrombocytopenic Purpura|journal=New England Journal of Medicine|volume=354|issue=18|year=2006|pages=1927–1935|issn=0028-4793|doi=10.1056/NEJMcp053024}}</ref>
| rowspan="7" |Thrombocytopenia
| align="left" style="background:#F5F5F5;" | History of:
|
*[[Cancer]]
==== Infection-Induced Thrombocytopenia ====
*[[Bone marrow transplantation]]
<span name="harr_c115s002s001s002p001"></span><span name="9100754"></span>
*[[Pregnancy]]
|
*[[Medication]]
**[[Platelet]] aggregation inhibitors ([[ticlopidine]] and [[clopidogrel]])
**Immunosuppressants ([[cyclosporine]], [[mitomycin]], [[tacrolimus]]/FK506, [[interferon|interferon-α]])
*[[HIV-1]] infection
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | −
|-
|-
|
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemolytic-uremic syndrome|Hemolytic Uremic Syndrome]]<ref name="Noris2005">{{cite journal|last1=Noris|first1=M.|title=Hemolytic Uremic Syndrome|journal=Journal of the American Society of Nephrology|volume=16|issue=4|year=2005|pages=1035–1050|issn=1046-6673|doi=10.1681/ASN.2004100861}}</ref>
==== Drug-Induced Thrombocytopenia ====
| align="left" style="background:#F5F5F5;" |History of:
<span name="harr_c115s002s001s003p001"></span><span name="9100757"></span>
* [[Infections]]
|
* [[Malignancy]], [[chemotherapy]], and [[ionizing radiation]]
* [[Calcineurin inhibitor]]s and [[transplantation]]
* [[Pregnancy]], [[HELLP syndrome]], and [[oral contraceptive pill]]
* [[Systemic lupus erythematosis]] 
* [[Antiphospholipid syndrome|Antiphospholipid antibody syndrome]]
* [[Glomerulopathy]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | −
|-
|-
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory
==== Heparin-Induced Thrombocytopenia ====
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
<span name="harr_c115s002s001s004p001"></span><span name="9100761"></span>
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments
|-
|-
|
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |Thromobcytosis
==== Immune Thrombocytopenic Purpura (ITP) ====
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Iron deficiency anemia|Iron deficiency anemia]]
<span name="harr_c115s002s001s005p001"></span><span name="9100771"></span>
Inflammatory diseases
|
 
[[Splenectomy]]
 
[[Essential thrombocytosis|Essential thrombocytosis]]
| align="left" style="background:#F5F5F5;" |
* Digital [[pain]]
* [[Gangrene]]
* [[Erythromelalgia]]
* H[[eadache]]
* [[Paresthesia|Paresthesias]] 
* [[Transient ischemic attack|Transient ischemic attacks]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl or ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | −
|-
|-
|
! rowspan="6" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Qualitative Disorders of [[Platelet]] Function
==== Inherited Thrombocytopenia ====
! rowspan="4" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited Disorders of [[Platelet]] Function
<span name="harr_c115s002s001s006p001"></span><span name="9100783"></span>
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Glanzmann's thrombasthenia|Glanzmann’s thrombasthenia]]
|
| align="left" style="background:#F5F5F5;" |
* Positive family history
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | Rare
| align="center" style="background:#F5F5F5;" | Nl or ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="left" style="background:#F5F5F5;" |
* AR inheritance
* Absence of the platelet Gp IIb/IIIa receptor
* Diminished for GP 2B-3A on [[Flow cytometry|flow cytometry]]
|-
|-
|
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Bernard-Soulier syndrome]]
==== Thrombotic Thrombocytopenic Purpura (TTP) ====
| align="left" style="background:#F5F5F5;" |
<span name="harr_c115s002s002s001p001"></span><span name="9100787"></span>
* Positive family history
|
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | Nl or ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="left" style="background:#F5F5F5;" |
* AR inheritance
* Absence of the platelet Gp Ib-IX-V receptor
* On PBS: giant platelets
* Ristocetin - no aggregation
|-
|-
|
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Wiskott-Aldrich syndrome]]
==== Hemolytic Uremic Syndrome ====
| align="left" style="background:#F5F5F5;" |
<span name="harr_c115s002s002s002p001"></span><span name="9100796"></span>
* Positive family history
|
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | Nl or ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="left" style="background:#F5F5F5;" |
* Anti-WASP antibody can be used to detect presence or absence of WAS protein
* In Wiskott–Aldrich syndrome, the [[Platelet|platelets]] are small and do not function properly. They are removed by the [[spleen]], which leads to low [[platelet]] counts.
|-
|-
|
! align="left" style="padding: 5px 5px; background: #DCDCDC;" |Platelet storage pool disorder:
=== Thromobcytosis ===
*[[Hermansky-Pudlak syndrome]]
<span name="harr_c115s002s003p001"></span><span name="9100800"></span>
*[[Chediak-Higashi syndrome]]
|Thrombocytosis in response to acute or chronic inflammation has not been associated with an increased thrombotic risk. In fact, patients with markedly elevated platelet counts (>1.5 million), usually seen in the setting of a myeloproliferative disorder, have an increased risk of bleeding. This appears to be due, at least in part, to acquired von Willebrand disease (VWD) due to platelet-VWF adhesion and removal.
*[[Gray platelet syndrome]]
|
| align="left" style="background:#F5F5F5;" |
* Positive family history
* [[Hairy cell leukemia]]
* [[Cardiovascular bypass]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | Nl or
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="left" style="background:#F5F5F5;" |
* AD inheritance
* Abnormlities of platelet granule formation
|-
|-
|
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Acquired Disorders of [[Platelet]] Function
|
| align="left" style="background:#F5F5F5 " |
==== Qualitative Disorders of Platelet Function ====
* [[Chronic renal failure pathophysiology|Uremia]]
<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
* [[Cardiopulmonary bypass]]
|
* Hematologic disorders such as: [[Myeloproliferative disease|myeloproliferative]] and [[Myelodysplastic syndrome|myelodysplastic syndromes]]
==== Inherited Disorders of Platelet Function ====
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
|
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" | Nl or ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | −
|-
|-
|
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Von Willebrand disease|Von Willebrand Disease]]
|
| align="left" style="background:#F5F5F5;" |
|
* Easy bruising
==== Acquired Disorders of Platelet Function ====
* [[Epistaxis]]
<span name="harr_c115s002s004s002p001"></span><span name="9100808"></span>
* Oral cavity bleeding
|
* Bleeding after dental extraction/surgery
* [[Menorrhagia]]
* [[Postpartum hemorrhage]]
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | See the table below for the details about different types.
|-
|-
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory
|
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
=== von Willebrand Disease ===
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding
<span name="harr_c115s002s005p001"></span><span name="9100810"></span>
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments
|-
|-
|
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Vessel wall disorders
|
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Metabolism|Metabolic]] and [[Inflammation|Inflammatory]] Disorders
|
! colspan="2" align="left" style="padding: 5px 5px; background: #DCDCDC;" |
|
* Acute febrile illnesses
* [[Cryoglobulinemia|Mixed cryoglobulinemia]]
* [[Monoclonal gammopathy|Monoclonal gammopathies]]
* [[Rocky Mountain spotted fever]]
* [[Vitamin C deficiency]]
* [[Cushing's syndrome|Cushing’s syndrome]]
* Chronic [[glucocorticoid]] therapy
* [[Vasculitis]] such as Henoch-Schönlein Purpura
| align="left" style="background:#F5F5F5;" |
* History of the underlying disease
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl or ↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |
|-
|-
|
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited Disorders of the [[Vessel wall|Vessel Wall]]
|
! colspan="2" align="left" style="background:#DCDCDC;" |
|
* [[Marfan's syndrome|Marfan’s syndrome]]
|
* [[Ehlers-Danlos syndrome]]
* [[Pseudoxanthoma elasticum]]
* [[Hereditary hemorrhagic telangiectasia]] ([[Hereditary hemorrhagic telangiectasia|HHT]], or [[Osler-Weber-Rendu|Osler-Weber-Rendu disease]])
| align="left" style="background:#F5F5F5;" |
* Positive family history
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl or ↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |−
|-
|-
|
! rowspan="15" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Coagulation factor disorders
|
<ref name="pmid28966616" />
|
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Fibrinogen]] deficiency
|
! colspan="2" align="left" style="padding: 5px 5px; background: #DCDCDC;" |
Different types of the [[fibrinogen]] disorders:
* [[Fibrinogen#Congenital afibrinogenemia|Congenital afibrinogenemia]]
* [[Fibrinogen#Congenital hypofibrinogenemia|Congenital hypofibrinogenemia]]
* [[Fibrinogen#Fibrinogen storage disease|Fibrinogen storage disease]]
* [[Fibrinogen#Congenital dysfibrinogenemia|Congenital dysfibrinogenemia]]
* [[Fibrinogen#Hereditary fibrinogen A.CE.B1-Chain amyloidosis|Hereditary fibrinogen Aα-Chain amyloidosis]]
* [[Fibrinogen#Acquired dysfibrinogenemia|Acquired dysfibrinogenemia]]
| align="left" style="background:#F5F5F5;" |
* [[Epistaxis]]
* Easy [[Bruise|bruising]]
* [[Menorrhagia]]
* [[Muscle]] bleeds
* [[Hemarthrosis]]
* [[Bleeding]] from the [[umbilical cord]] stump after birth
* Bleeding after [[dental surgery]] or tooth extraction
* AbNl bleeding during or after injury, surgery, or childbirth
* [[Gastrointestinal tract|Gastrointestinal]] [[hemorrhage]]
* [[Cerebral hemorrhage]]
* [[Thrombosis]]
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="left" style="background:#F5F5F5;" |
* Impaired fibrin cross-linking or clot dissolution
* Mild or severe bleeding depend on levels of functional fibrinogen
* Variable age of onset
|-
|-
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory
|
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments
|-
|-
| rowspan="2" |Vessel wall disorders
! colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Prothrombin deficiency]]
|Metabolic and Inflammatory Disorders
| align="left" style="background:#F5F5F5;" |  
|
* [[Epistaxis]]
|
* Soft-tissue hemorrhage
* Excessive postoperative bleeding
* [[Menorrhagia]]
* Muscle [[Hematoma|hematomas]]
* [[Hemarthrosis]]
* [[Intracranial hemorrhage|Intracranial]] bleeding
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | Nl
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | −
|-
|-
|Inherited Disorders of the Vessel Wall
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor V deficiency]]
|
| align="left" style="background:#F5F5F5;" |
|
* Excessive bruising with minor injuries
* [[Epistaxis]]
* [[Hemarthrosis]]
* [[Menorrhagia]]
* [[Intracerebral hemorrhage|Intracerebral hemorrhages]]
* [[Pulmonary hemorrhage]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |
* The severity of bleeding related to the degree of factor V deficiency
|-
|-
| rowspan="12" |Coagulation disorders
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor VII deficiency]]
|Fibrinogen
| align="left" style="background:#F5F5F5;" |
|
* Easy [[Bruise|bruising]]
|
* Mucosal bleeding
* Postoperative bleeding
* [[Menorrhagia]]
* Soft tissue hematomas
* [[Thrombosis]]
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="left" style="background:#F5F5F5;" |
* Thrombosis in inherited factor VII deficiency
* Treatment with the administration of factor VII replacement therapy
|-
|-
|Prothrombin
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor X deficiency]]
|
| align="left" style="background:#F5F5F5;" |
|
* Prolonged bleeding following circumcision
* Easy [[Bruise|bruising]]
* [[Hematuria]]
* [[Menorrhagia]]
* Abortion
* Postpartum hemorrhage
* Epistaxis
* Pseudotumors
* Intracranial bleeding
* Hemarthroses
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |−
|-
|-
| rowspan="1" |Factor V
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor XII|Factor XII deficiency]]
|
| align="left" style="background:#F5F5F5;" |
|
* Asymptomatic
* Recurrent miscarriages
* Painful leg ulcers
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |
|-
|-
| rowspan="1" |Factor VII
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory
|
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments
|-
|-
| rowspan="1" |Factor VIII
! colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[High-molecular-weight kininogen|High molecular weight kininogen (HMWK)]] deficiency
|
| align="left" style="background:#F5F5F5;" |
|
* Positive family history of bleeding
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |
|-
|-
| rowspan="1" |Factor IX
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Prekallikrein]] deficiency
|
| align="left" style="background:#F5F5F5;" |
|
* Positive family history of bleeding
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |
|-
|-
| rowspan="1" |Factor X
! rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor XIII deficiency]]<ref>{{cite journal | vauthors =  Dorgalaleh A, Naderi M, Hosseini MS, Alizadeh S, Hosseini S, Tabibian S |display-authors=etal | title = Factor XIII Deficiency in Iran: A Comprehensive Review of the Literature. Seminars in thrombosis and hemostasis;  |  volume = 41  | issue =  3 (41) | pages = 323–329 | date=2015}}</ref>
|
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |
|
* Sub unit A mutation disease (more common)
* Sub unit B mutation disease
| align="left" style="background:#F5F5F5;" |
* Positive family history of bleeding
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |±
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl or ↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="left" style="background:#F5F5F5;" |
* Impaired fibrin cross-linking or clot dissolution
* The severity of factor XIII deficiency bleeds can be different in different patients
|-
|-
| rowspan="1" |Factor XI
! rowspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemophilia]]<ref name="pmid26897598">{{cite journal |vauthors=Peyvandi F, Garagiola I, Young G |title=The past and future of haemophilia: diagnosis, treatments, and its complications |journal=Lancet |volume=388 |issue=10040 |pages=187–97 |date=July 2016 |pmid=26897598 |doi=10.1016/S0140-6736(15)01123-X |url=}}</ref>
|
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type A deficiency
|
| align="left" style="background:#F5F5F5;" |
* Eeasy [[Bruise|bruising]]
* Inadequate clotting in [[trauma]] or mild injury
* Spontaneous hemorrhage
* [[Hemarthrosis]]
* [[Epistaxis]]
* [[Gingival bleeding]]
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |−
|-
|-
| rowspan="1" |Factor XII
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type B deficiency
|
| align="left" style="background:#F5F5F5;" |
|
* Neonatal bleeding
* Trauma-related soft tissue hemorrhage
* [[Hemarthrosis]] 
* [[Hematoma|Hematomas]]
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |−
|-
|-
|HK
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type C deficiency
|
| align="left" style="background:#F5F5F5;" |
|
* Positive family history
* Bleeding after surgery or injury
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |Rare
| align="center" style="background:#F5F5F5;" |Rare
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |−
|-
|-
| rowspan="1" |Prekallikrein
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory
|
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments
|-
|-
| rowspan="1" |Factor XIII
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Rare diseases
|
! colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Disseminated intravascular coagulation|Disseminated Intravascular Coagulation]]<ref name="pmid580488">{{cite journal |vauthors=Siegal T, Seligsohn U, Aghai E, Modan M |title=Clinical and laboratory aspects of disseminated intravascular coagulation (DIC): a study of 118 cases |journal=Thromb. Haemost. |volume=39 |issue=1 |pages=122–34 |date=February 1978 |pmid=580488 |doi= |url=}}</ref><ref name="pmid19222477">{{cite journal |vauthors=Levi M, Toh CH, Thachil J, Watson HG |title=Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology |journal=Br. J. Haematol. |volume=145 |issue=1 |pages=24–33 |date=April 2009 |pmid=19222477 |doi=10.1111/j.1365-2141.2009.07600.x |url=}}</ref><ref name="pmid18066597">{{cite journal |vauthors=Ghosh K, Shetty S |title=Blood coagulation in falciparum malaria--a review |journal=Parasitol. Res. |volume=102 |issue=4 |pages=571–6 |date=March 2008 |pmid=18066597 |doi=10.1007/s00436-007-0832-0 |url=}}</ref><ref name="pmid5804882">{{cite journal |vauthors=Siegal T, Seligsohn U, Aghai E, Modan M |title=Clinical and laboratory aspects of disseminated intravascular coagulation (DIC): a study of 118 cases |journal=Thromb. Haemost. |volume=39 |issue=1 |pages=122–34 |date=February 1978 |pmid=580488 |doi= |url=}}</ref><ref name="pmid1531791">{{cite journal |vauthors=Fourrier F, Chopin C, Goudemand J, Hendrycx S, Caron C, Rime A, Marey A, Lestavel P |title=Septic shock, multiple organ failure, and disseminated intravascular coagulation. Compared patterns of antithrombin III, protein C, and protein S deficiencies |journal=Chest |volume=101 |issue=3 |pages=816–23 |date=March 1992 |pmid=1531791 |doi= |url=}}</ref>
|
| align="left" style="background:#F5F5F5;" |
* [[Trauma]]
* [[Burn]]
* [[Crush injury]]
* [[Sepsis]]
* [[Malignancy]]
* Obstetric complication: abruption, amniotic fluid embolism
* [[Hemolytic anemia]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |↓
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |−
|-
|-
| rowspan="3" |[[Hemophilia]]
! colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Vitamin K Deficiency]]<ref name="pmid165052572">{{cite journal| author=Dezee KJ, Shimeall WT, Douglas KM, Shumway NM, O'malley PG| title=Treatment of excessive anticoagulation with phytonadione (vitamin K): a meta-analysis. | journal=Arch Intern Med | year= 2006 | volume= 166 | issue= 4 | pages= 391-7 | pmid=16505257 | doi=10.1001/.391 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16505257  }}</ref>
|Type A
| align="left" style="background:#F5F5F5;" |
|
* Bleeding after trauma
|
* [[Epistaxis]]
* [[Hematoma]]
* Gastrointestinal bleeding
* [[Menorrhagia]]  
* [[Hematuria]]
* Gum bleeding
* Oozing from venipuncture sites
* Easy [[Bruise|bruisability]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |−
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |↑
| align="center" style="background:#F5F5F5;" |Nl or mildly prolonged
| align="center" style="background:#F5F5F5;" |Nl
| align="center" style="background:#F5F5F5;" |−
|}
 
==Different types of Von-Willebrand diseases==
{|
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Type of VWD
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Type of factor deficiency
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Prevalence
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Inheritance pattern
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Clinical manifestations
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |VWF activity
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |RIPA
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Factor VIII
|-
|-
|Type B
! colspan="2" align="center" style="background:#DCDCDC;" |Type 1
|
| align="center" style="background:#F5F5F5;" | Quantitative/ partial
|
| align="center" style="background:#F5F5F5;" | 60-70%
| align="center" style="background:#F5F5F5;" | AD
| align="left" style="background:#F5F5F5;" |  
* Bleeding severity mild to severe
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" |
|-
|-
|Type C
! rowspan="4" align="center" style="background:#DCDCDC;" |Type 2
|
! align="center" style="background:#DCDCDC;" |2A
|
| align="center" style="background:#F5F5F5;" | Qualitative
| align="center" style="background:#F5F5F5;" | 10%
| align="center" style="background:#F5F5F5;" | AD/AR
| align="left" style="background:#F5F5F5;" |
* Moderate to severe [[bleeding]]
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | N or ↓
|-
|-
| rowspan="4" |Rare diseases
! align="center" style="background:#DCDCDC;" |2B
|Disseminated Intravascular Coagulation
| align="center" style="background:#F5F5F5;" | Qualitative
|
| align="center" style="background:#F5F5F5;" | 5%
|
| align="center" style="background:#F5F5F5;" | AD
| align="left" style="background:#F5F5F5;" |
* [[Thrombocytopenia]]
* Moderate to severe [[bleeding]]
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | N or ↓
|-
|-
|Vitamin K Deficiency
! align="center" style="background:#DCDCDC;" |2M
|
| align="center" style="background:#F5F5F5;" | Qualitative
|
| align="center" style="background:#F5F5F5;" | <1%
| align="center" style="background:#F5F5F5;" | AD/AR
| align="left" style="background:#F5F5F5;" |  
* Moderate to severe [[bleeding]]
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | N or ↓
|-
|-
|Coagulation Disorders Associated with Liver Failure
! align="center" style="background:#DCDCDC;" |2N
|
| align="center" style="background:#F5F5F5;" | Qualitative
|
| align="center" style="background:#F5F5F5;" | <1%
| align="center" style="background:#F5F5F5;" | AR
| align="left" style="background:#F5F5F5;" |
* Clinically similar to [[hemophilia A]] with [[joint]], [[soft tissue]], [[urinary]] [[bleeding]]
| align="center" style="background:#F5F5F5;" | N
| align="center" style="background:#F5F5F5;" | N
| align="center" style="background:#F5F5F5;" | ↓
|-
|-
|Acquired Inhibitors of Coagulation Factors
! colspan="2" align="center" style="background:#DCDCDC;" |Type 3
|
| align="center" style="background:#F5F5F5;" | Complete deficiency
|
| align="center" style="background:#F5F5F5;" | 1-2%
| align="center" style="background:#F5F5F5;" | AR
| align="left" style="background:#F5F5F5;" |
* Clinically similar to [[hemophilia A]] with [[joint]] and [[soft tissue]] [[bleeding]]
* Severe [[mucosal bleeding]]
| align="center" style="background:#F5F5F5;" | Absent
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | Low, 1-10%
|}
|}
For more information on Von Willebrand disease, click [[ von willebrand disease | here]].
==References==
{{Reflist|2}}
[[Category:Medicine]]
[[Category:Hematology]]
[[Category:Oncology]]
[[Category:Up-To-Date]]
[[Category:Emergency medicine]]

Latest revision as of 20:38, 29 July 2020


Bleeding diathesis main page

Overview

Classification

Differential Diagnosis

Platelet disorders
Immune Thrombocytopenic Purpura
Thrombotic Thrombocytopenic Purpura
Hemolytic Uremic Syndrome
Thrombocytosis
Von Willebrand Disease
Coagulation disorders
Fibrinogen deficiency
Prothrombin deficiency
Factor V deficiency
Factor VII deficiency
Factor VIII deficiency
Factor IX deficiency
Factor X deficiency
Factor XI deficiency
Factor XII deficiency
High-molecular-weight kininogen deficiency
Prekallikrein deficiency
Factor XIII deficiency
Hemophilia
Rare diseases
Disseminated Intravascular Coagulation
Vitamin K Deficiency

Different types of Von-Willebrand diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2], Nazia Fuad M.D., Sogand Goudarzi, MD [3]

Synonyms and keywords: Hypocoagulopathy; blood coagulation disorders; hemorrhagic diathesis; hemostasis abnormality; bleeding tendency


Overview

Bleeding diathesis is susceptibility to bleed due to coagulopathy disorders or platelets disorders. These diseases can occur due to a disorder of homeostasis, localized process (tissue injury), or medications. Bleeding diathesis can be resulted from vessel wall injury, platelet disorders, and coagulation factor disorders. Clinical manifestation of bleeding disorders can have a wide range of symptoms from asymptomatic to symptomatic massive and life threatening bleeding. Platelet disorders mostly have skin manifestations such as petechiae, and ecchymoses. In order to find the cause of hypo-coagulopathy; there are established laboratory tests, such as peripheral blood smear, platelet count and platelet function analysis, coagulation factor deficiencies and inhibitors, fibrinolysis tests (eg. D-dimer level), bleeding time (BT), prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time (TT), and reptilase time. In the case of any abnormal bleeding, first line of screening tests are CBC, PT, PTT, BT, and TT.

Classification

Disorders of hemostasis can be classified into two main categories: platelet disorders, and disorders of coagulation. Each category can be further classified as bellow:

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Abnormal hemostasis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

• Patient history-sign & symptom: deep soft tissue & mucocutaneus bleeding
• Screen test CBC-Plt-PT&PTT-BT-TT
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hx of deep soft tissue bleeding
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hx of mucocotaneus bleeding
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Coagulopathy
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Plt disorder
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Family history
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal plt count
 
 
Low plt count
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
(+)
• Inherited coagulpathy
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
(-)
• Acquired coagulopathy
 
 
 
 
 
 
Functional Plt disorder
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
↑Plt
• Hemophillia
• VWD
• Factor VIII or IX deficiency
 
↑PT
• Factor VII deficiency
 
↑PT&↑PTT
• Fibrinogen deficiency
• Factor II deficiency
• Factor V deficiency
• Factor X deficiency
 
 
↑PTT
• Factor inhibitor
• Anti phospholipid Ab syndrome
 
↑PT
• Factor inhibitor
• Vit K deficiency
• Liver disease
 
↑PT&↑PTT
• Factor inhibit
• DIC
• Liver failure
• Late stage of Vit K deficiency
 
↑ Afibrinogenemia
• Heparin inhibitor
• Direct thrombin inhibitor
 
 
 
 
Abnormal solobity
• Factor XIII deficiency
• Cross-linkin inhibitor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Not corrected with mixing with NL plasma
 
 
 
 
 
 
 
 
HX(+)
 
HX(-)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

• Factor inhibitors
• Lupus anti coagulant
• DIC
• Heparin or direct thrombin inhibitors
 
 
 
 
 
 
 
 
Congenital
 
Acquired
 
 
 
 
 

Differentiating Bleeding Disorders from Other Diseases

Different causes of bleeding disorders can be differentiated based on their clinical manifestation and laboratory findings.

These features have discussed in the below table:

Category Subcategory Disease History Clinical manifestation Laboratory testing Comments
Mucosal bleeding Petechia Ecchymoses Menorrhagia Hematoma Hemarthrosis Plt BT PT PTT TT
Platelet disorders Thrombocytopenia Infection-Induced thrombocytopenia[1]
  • History of prior infection
+ + + + + + Nl Nl Nl
Medication-Induced Thrombocytopenia[2] + + + + + + Nl Nl Nl Most important part of treatment is discontinuing of the medication.
Heparin-Induced thrombocytopenia[3] + + + + + + Nl Nl For more information click here: Heparin-induced thrombocytopenia.
Immune Thrombocytopenic Purpura[4] + + + + + + Nl Nl Nl
Inherited Thrombocytopenia[5]
  • Positive family history
+ + + + + + Nl Nl Nl
Thrombotic Thrombocytopenic Purpura[6] History of: + + + + + + Nl Nl Nl
Hemolytic Uremic Syndrome[7] History of: + + + + + + Nl Nl Nl
Subcategory Disease History Mucosal bleeding Petechia Ecchymoses Menorrhagia Hematoma Hemarthrosis Plt BT PT PTT TT Comments
Thromobcytosis Iron deficiency anemia

Inflammatory diseases

Splenectomy

Essential thrombocytosis

± ± Nl or ↑ Nl Nl Nl
Qualitative Disorders of Platelet Function Inherited Disorders of Platelet Function Glanzmann’s thrombasthenia
  • Positive family history
+ + + + Rare Nl or ↓ Nl Nl Nl
  • AR inheritance
  • Absence of the platelet Gp IIb/IIIa receptor
  • Diminished for GP 2B-3A on flow cytometry
Bernard-Soulier syndrome
  • Positive family history
+ + + + Nl or ↓ Nl Nl Nl
  • AR inheritance
  • Absence of the platelet Gp Ib-IX-V receptor
  • On PBS: giant platelets
  • Ristocetin - no aggregation
Wiskott-Aldrich syndrome
  • Positive family history
+ + + + Nl or ↓ Nl Nl Nl
  • Anti-WASP antibody can be used to detect presence or absence of WAS protein
  • In Wiskott–Aldrich syndrome, the platelets are small and do not function properly. They are removed by the spleen, which leads to low platelet counts.
Platelet storage pool disorder: + + + + Nl or ↓ Nl Nl Nl
  • AD inheritance
  • Abnormlities of platelet granule formation
Acquired Disorders of Platelet Function + + + + ± ± Nl or ↓ Nl Nl Nl
Von Willebrand Disease + + + + ± ± Nl Nl See the table below for the details about different types.
Subcategory Disease History Mucosal bleeding Petechia Ecchymoses Menorrhagia Hematoma Hemarthrosis Plt BT PT PTT TT Comments
Vessel wall disorders Metabolic and Inflammatory Disorders
  • History of the underlying disease
+ + ± Nl Nl or ↑ Nl Nl Nl
Inherited Disorders of the Vessel Wall
  • Positive family history
+ + ± Nl Nl or ↑ Nl Nl Nl
Coagulation factor disorders

[8]

Fibrinogen deficiency

Different types of the fibrinogen disorders:

+ + ± + Nl
  • Impaired fibrin cross-linking or clot dissolution
  • Mild or severe bleeding depend on levels of functional fibrinogen
  • Variable age of onset
Subcategory Disease History Mucosal bleeding Petechia Ecchymoses Menorrhagia Hematoma Hemarthrosis Plt BT PT PTT TT Comments
Prothrombin deficiency + + + + + Nl Nl
Factor V deficiency + + + + Nl Nl
  • The severity of bleeding related to the degree of factor V deficiency
Factor VII deficiency + + + Nl Nl Nl
  • Thrombosis in inherited factor VII deficiency
  • Treatment with the administration of factor VII replacement therapy
Factor X deficiency
  • Prolonged bleeding following circumcision
  • Easy bruising
  • Hematuria
  • Menorrhagia
  • Abortion
  • Postpartum hemorrhage
  • Epistaxis
  • Pseudotumors
  • Intracranial bleeding
  • Hemarthroses
+ + + + + Nl Nl Nl
Factor XII deficiency
  • Asymptomatic
  • Recurrent miscarriages
  • Painful leg ulcers
Nl Nl Nl Nl
Subcategory Disease History Mucosal bleeding Petechia Ecchymoses Menorrhagia Hematoma Hemarthrosis Plt BT PT PTT TT Comments
High molecular weight kininogen (HMWK) deficiency
  • Positive family history of bleeding
Nl Nl Nl Nl
Prekallikrein deficiency
  • Positive family history of bleeding
Nl Nl Nl Nl
Factor XIII deficiency[9]
  • Sub unit A mutation disease (more common)
  • Sub unit B mutation disease
  • Positive family history of bleeding
± ± ± ± ± ± Nl Nl Nl or ↑ Nl Nl
  • Impaired fibrin cross-linking or clot dissolution
  • The severity of factor XIII deficiency bleeds can be different in different patients
Hemophilia[10] Type A deficiency + + + Nl Nl Nl Nl
Type B deficiency + + + Nl Nl Nl Nl
Type C deficiency
  • Positive family history
  • Bleeding after surgery or injury
+ Rare Rare Nl Nl Nl Nl
Subcategory Disease History Mucosal bleeding Petechia Ecchymoses Menorrhagia Hematoma Hemarthrosis Plt BT PT PTT TT Comments
Rare diseases Disseminated Intravascular Coagulation[11][12][13][14][15] + + + + + + Nl
Vitamin K Deficiency[16] + + + + + Nl Nl or mildly prolonged Nl

Different types of Von-Willebrand diseases

Type of VWD Type of factor deficiency Prevalence Inheritance pattern Clinical manifestations VWF activity RIPA Factor VIII
Type 1 Quantitative/ partial 60-70% AD
  • Bleeding severity mild to severe
Type 2 2A Qualitative 10% AD/AR N or ↓
2B Qualitative 5% AD N or ↓
2M Qualitative <1% AD/AR N or ↓
2N Qualitative <1% AR N N
Type 3 Complete deficiency 1-2% AR Absent Low, 1-10%

For more information on Von Willebrand disease, click here.

References

  1. Herbinger, K.-H.; Schunk, M.; Nothdurft, H. D.; von Sonnenburg, F.; Löscher, T.; Bretzel, G. (2012). "Comparative study on infection-induced thrombocytopenia among returned travellers". Infection. 40 (4): 373–379. doi:10.1007/s15010-012-0242-9. ISSN 0300-8126.
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