Autoimmune hemolytic anemia differential diagnosis: Difference between revisions

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__NOTOC__
__NOTOC__
{{Autoimmune hemolytic anemia}}
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Autoimmune_hemolytic_anemia]]


{{CMG}} '''Assosciate Editor(s)-In-Chief:''' [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]; {{shyam}}, [[User:Irfan Dotani|Irfan Dotani]] [3]
{{CMG}} '''Assosciate Editor(s)-In-Chief:''' [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]; {{shyam}}, [[User:Irfan Dotani|Irfan Dotani]] [3]
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==Differentiating Autoimmune hemolytic anemia from other Diseases==
==Differentiating Autoimmune hemolytic anemia from other Diseases==
=== Differential diagnosis of "Bleeding disorders": ===
 
<span name="harr_c115s002s003p001"></span><span name="9100800"></span><span name="harr_c115s002s003p001"></span><span name="9100800"></span>
{| class="wikitable"
{| class="wikitable" align="center" style="border: 0px; font-size: 90%; margin: 3px;"
|+
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category
! rowspan="2" |Type of anemia
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Sub-category
! rowspan="2" |MCV
! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Diseases
! rowspan="2" |Hemolysis
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! rowspan="2" |Intrinsic/Extrinsic
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical manifestation
! rowspan="2" |Hb concentration
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory testing
! rowspan="2" |RDW
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments
! rowspan="2" |Reticulocytosis
! rowspan="2" |Haptoglobin levels
! rowspan="2" |Specific Symptoms
! rowspan="2" |Specific History
! rowspan="2" |Physical examination
! rowspan="2" |Genetics
!
! colspan="5" |Iron studies
! rowspan="2" |Specific finding on blood smear
|-
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |<nowiki>Petechia|Petechiae</nowiki>
!Hepcidin
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
!Serum iron
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
!Serum Tfr level
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma
!Transferrin or TIBC
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis
!Ferritin
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Platelet count
!% Transferrin saturation
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Bleeding time (BT)
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Prothrombin time (PT)
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Activated partial thromboplastin time (aPTT)
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Thrombin time (TT)
|-
|-
| rowspan="13" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Platelet]] disorders
!Autoimmune  hemolytic anemia
| rowspan="7" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombocytopenia]]
|
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Infection]]-Induced thrombocytopenia<span name="harr_c115s002s001s002p001"></span><span name="9100754"></span><ref name="pmid21325604">{{cite journal |vauthors=Neunert C, Lim W, Crowther M, Cohen A, Solberg L, Crowther MA |title=The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia |journal=Blood |volume=117 |issue=16 |pages=4190–207 |date=April 2011 |pmid=21325604 |doi=10.1182/blood-2010-08-302984 |url=}}</ref><ref name="pmid26906627">{{cite journal |vauthors=Karimi O, Goorhuis A, Schinkel J, Codrington J, Vreden SGS, Vermaat JS, Stijnis C, Grobusch MP |title=Thrombocytopenia and subcutaneous bleedings in a patient with Zika virus infection |journal=Lancet |volume=387 |issue=10022 |pages=939–940 |date=March 2016 |pmid=26906627 |doi=10.1016/S0140-6736(16)00502-X |url=}}</ref><ref name="pmid25600600">{{cite journal |vauthors=Zammarchi L, Stella G, Mantella A, Bartolozzi D, Tappe D, Günther S, Oestereich L, Cadar D, Muñoz-Fontela C, Bartoloni A, Schmidt-Chanasit J |title=Zika virus infections imported to Italy: clinical, immunological and virological findings, and public health implications |journal=J. Clin. Virol. |volume=63 |issue= |pages=32–5 |date=February 2015 |pmid=25600600 |doi=10.1016/j.jcv.2014.12.005 |url=}}</ref>
* [[Normocytic anemia|Normocytic]]
|
* + ([[Intravascular]])
|
* Extrinsic
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Painful blue fingers and toes on exposure to cold temperature.
* [[Chest pain]]
* [[Rigor|Chills]]
* [[Dizziness]]
* [[Tachycardia]]
* [[Headache]]
* [[Fatigue]]
|Associated with:
* SLE
* CLL
* Mycoplasia pneumoniae infection
|
* Painful, blue fingers and toes with cold weather
|<nowiki>--</nowiki>
|
* Normal
|
* ↓(Decreased)
|
* Normal
|<nowiki>--</nowiki>
|<nowiki>--</nowiki>
|<nowiki>--</nowiki>
|
|
* History of prior infection
* RBC agglutination
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
|<nowiki>-</nowiki>
|-
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Medication|Medications]]-Induced thrombocy<span name="harr_c115s002s001s003p001"></span><span name="9100757"></span>topenia <ref name="pmid25134884">{{cite journal |vauthors=Kam T, Alexander M |title=Drug-induced immune thrombocytopenia |journal=J Pharm Pract |volume=27 |issue=5 |pages=430–9 |date=October 2014 |pmid=25134884 |doi=10.1177/0897190014546099 |url=}}</ref><ref name="pmid23461497">{{cite journal |vauthors=Seco-Melantuche R, Delgado-Sánchez O, Álvarez-Arroyo L |title=[Incidence of drug-induced thrombocytopenia in hospitalized patients] |language=Spanish; Castilian |journal=Farm Hosp |volume=37 |issue=1 |pages=27–34 |date=2013 |pmid=23461497 |doi=10.7399/FH.2013.37.1.42 |url=}}</ref>
!Iron deficiency anemia
|
* [[Microcytic anemia|Microcytic]]
| --
| --
|
* [[Hypochromic anemia|Hypochromic]]
|
* ↑(Increased)
|
* Normal or ↓(Decreased)
|
* Normal
|
* [[Koilonychia]]
* [[Pica]]
|
* [[Menorrhagia]]
* [[GI]] loss
* [[Gastrointestinal tract|GI]] surgery
* [[Pregnancy]]
|
* [[Glossitis]]
* [[Cheilosis]]
* [[Dysphagia]]
| --
|
* Normal
|
* ↓(Decreased)
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* ↓↓↓(Decreased)
|
|
*History of [[Medication|medications]] such as:
* Central [[pallor]]
** [[Furosemide]]
** [[Non-steroidal anti-inflammatory drug|Nonsteroidal anti-inflammatory drugs]] ([[Non-steroidal anti-inflammatory drug|NSAIDs]])
** [[Penicillin]]
** [[Quinidine]]
** [[Quinine]]
** [[Ranitidine]]
** [[Sulfonamide (medicine)|Sulfonamides]]
** [[Linezolid]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
|Most important par of treatment is discontinuing of the medication.
|-
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Heparin-Induced Thrombocytopenia|Heparin-Induced thrombocytopenia]]<span name="harr_c115s002s001s004p001"></span><span name="9100761"></span><ref name="pmid25629757">{{cite journal |vauthors=Warkentin TE, Safyan EL, Linkins LA |title=Heparin-induced thrombocytopenia presenting as bilateral adrenal hemorrhages |journal=N. Engl. J. Med. |volume=372 |issue=5 |pages=492–4 |date=January 2015 |pmid=25629757 |doi=10.1056/NEJMc1414161 |url=}}</ref>
!Lead poisoning
|
* [[Microcytic anemia|Microcytic]]
| --
| --
|
* [[Intravascular|Hypochromic]]
|
* Normal
|
* Normal or ↓(Decreased)
|
* Normal
|
* Burtonian lines
* [[Basophilic]] [[Stippling (dentistry)|stippling]]
* [[Wrist drop]]
* [[Foot drop]]
|
* House painted with chipped paint
|
* [[Wrist drop]]
* [[Foot drop]]
* Burtonian lines
|<nowiki>--</nowiki>
|
* Normal
|
* Normal or ↓(Decreased)
|
* Normal
|
* Normal
|
|
* [[Thrombosis]]
* Normal or ↓(Decreased)
* Unexplained [[thrombocytopenia]] up to 3 weeks after the end of [[heparin]] therapy
|<nowiki>--</nowiki>
|<nowiki>+</nowiki>
|
| +
* RBCs retain aggregates of [[Ribosomal RNA|rRNA]] (basophilic stippling)
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|↑
|For more information click here: [[Heparin-induced thrombocytopenia]].
|-
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Idiopathic thrombocytopenic purpura|Immune Thrombocytopenic Purpura]] ([[Idiopathic thrombocytopenic purpura|ITP]])<span name="harr_c115s002s001s005p001"></span><span name="9100771"></span><ref name="pmid8857953">{{cite journal |vauthors=Wright JF, Blanchette VS, Wang H, Arya N, Petric M, Semple JW, Chia WK, Freedman J |title=Characterization of platelet-reactive antibodies in children with varicella-associated acute immune thrombocytopenic purpura (ITP) |journal=Br. J. Haematol. |volume=95 |issue=1 |pages=145–52 |date=October 1996 |pmid=8857953 |doi= |url=}}</ref>
!Sideroblastic anemia
|
* [[Microcytic anemia|Microcytic]]
| --
| --
|
|
* History of prior [[infection]] or no history
* [[Hypochromic anemia|Hypochromic]]
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* Normal
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* Normal or ↓(Decreased)
|<nowiki>+</nowiki>
|
|
* Normal
|↑
|
|N
* [[Seborrheic dermatitis]]
|N
* Glossy Tongue
|N
* [[Tingling]]
| -
 
*
|
* [[Alcohol]] abuse most common cause
* [[Isoniazid]] use
* [[Chloramphenicol]] use
* [[Idiopathic]] (eg, refractory anemia with ringed [[Sideroblastic|sideroblasts]] [RARS], a [[Myelodysplastic syndrome|myelodysplastic]] syndrome)
|
* Patient present with symptoms of [[Vitamin B6]], [[copper deficiency]] symptoms
|
* Defect in [[ALA synthase]] gene
* [[Autosomal dominant]]
* [[Autosomal recessive]]
* [[X-linked]]
|
* Normal
|
* (Increased)
|
* Normal
|
* Normal or ↓(Decreased)
|
* ↑(Increased)
| --
|
* Ringed [[Sideroblastic|sideroblasts]]
|-
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited [[Thrombocytopenia]]<span name="harr_c115s002s001s006p001"></span><span name="9100783"></span><ref name="pmid27025194">{{cite journal |vauthors=Johnson B, Fletcher SJ, Morgan NV |title=Inherited thrombocytopenia: novel insights into megakaryocyte maturation, proplatelet formation and platelet lifespan |journal=Platelets |volume=27 |issue=6 |pages=519–25 |date=September 2016 |pmid=27025194 |pmc=5000870 |doi=10.3109/09537104.2016.1148806 |url=}}</ref><ref name="pmid30103613">{{cite journal |vauthors=Wang Q, Cao L, Sheng G, Shen H, Ling J, Xie J, Ma Z, Yin J, Wang Z, Yu Z, Chen S, Zhao Y, Ruan C, Xia L, Jiang M |title=Application of High-Throughput Sequencing in the Diagnosis of Inherited Thrombocytopenia |journal=Clin. Appl. Thromb. Hemost. |volume= |issue= |pages=1076029618790696 |date=August 2018 |pmid=30103613 |doi=10.1177/1076029618790696 |url=}}</ref>
!Anemia of chronic disease
|
* [[Microcytic anemia|Microcytic]]
| --
| --
|
* [[Hypochromic anemia|Hypochromic]]
|
* Normal
|
* Normal or ↓(Decreased)
|
* Normal
|
* [[Headache]]
* [[Shortness of breath]]
|
* [[Rheumatoid arthritis]]
* [[SLE]]
* [[Neoplasm]]
* [[Chronic kidney disease]]
|<nowiki>--</nowiki>
| --
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Normal
|
|
* Family history
* ↓(Decreased)
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* ↑(Increased)
|<nowiki>+</nowiki>
| --
|<nowiki>+</nowiki>
|<nowiki>--</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
| -
|-
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombotic thrombocytopenic purpura|Thrombotic Thrombocytopenic Purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]])<span name="harr_c115s002s002s001p001"></span><span name="9100787"></span><ref name="pmid30220931">{{cite journal |vauthors=Knöbl P |title=Thrombotic thrombocytopenic purpura |journal=Memo |volume=11 |issue=3 |pages=220–226 |date=2018 |pmid=30220931 |doi=10.1007/s12254-018-0429-6 |url=}}</ref><ref name="pmid26386489">{{cite journal |vauthors=Mannucci PM, Cugno M |title=The complex differential diagnosis between thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: Laboratory weapons and their impact on treatment choice and monitoring |journal=Thromb. Res. |volume=136 |issue=5 |pages=851–4 |date=November 2015 |pmid=26386489 |doi=10.1016/j.thromres.2015.09.007 |url=}}</ref>
!Thalassemia
|History of:
|
*[[Cancer]]
* [[Microcytic anemia|Microcytic]]
*[[Bone marrow transplantation]]
|<nowiki>--</nowiki>
*[[Pregnancy]]
| --
*[[Medication]]
|
**[[Platelet]] aggregation inhibitors ([[ticlopidine]] and [[clopidogrel]])
* [[Hypochromic anemia|Hypochromic]]
**Immunosuppressants ([[cyclosporine]], [[mitomycin]], [[tacrolimus]]/FK506, [[interferon|interferon-α]])
|
*[[HIV-1]] infection
* Normal
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* '''Thalassemia trait'''
|<nowiki>+</nowiki>
Normal or ↓(Decreased)
|<nowiki>+</nowiki>
* '''Thalassemia Syndromes'''
|<nowiki>+</nowiki>
(Increased)
|↓
|
|N
|N
|N
| -
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemolytic-uremic syndrome|Hemolytic Uremic Syndrome]]<span name="harr_c115s002s002s002p001"></span><span name="9100796"></span><ref name="pmid24365375">{{cite journal |vauthors=Webster K, Schnitzler E |title=Hemolytic uremic syndrome |journal=Handb Clin Neurol |volume=120 |issue= |pages=1113–23 |date=2014 |pmid=24365375 |doi=10.1016/B978-0-7020-4087-0.00075-9 |url=}}</ref><ref name="pmid25845294">{{cite journal |vauthors=Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P |title=Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome |journal=Pathol. Biol. |volume=63 |issue=3 |pages=136–43 |date=June 2015 |pmid=25845294 |doi=10.1016/j.patbio.2015.03.001 |url=}}</ref>
|History of:
* Infections


* [[Malignancy]], [[Cancer (disease)|cancer]] [[chemotherapy]] and [[ionizing radiation]]
|
* [[Calcineurin inhibitor]]<nowiki/>s and [[transplantation]]
* Normal
* [[Pregnancy]], [[HELLP syndrome]] and [[oral contraceptive pill]]
|'''α-thalassemia'''
* [[Systemic lupus erythematosis]] and [[Antiphospholipid syndrome|antiphospholipid antibody syndrome]]
* [[Hydrops fetalis]]
* [[Glomerulopathy]]
'''β-thalassemia'''
* [[Familial]], not included in part 1
* [[Skeletal]] deformities
|<nowiki>+</nowiki>
* Chipmunk facies
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* Associated with [[parvovirus B19]]  
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* [[Hepatomegaly]]
|
* [[Splenomegaly]]  
|
|'''α-thalassemia'''
|N
* '''''α'''''- globin gene deletions
|N
* [[Cis]] deletions
|N
* [[Trans]] deletions
| -
'''β-thalassemia'''
* [[Point mutation]] in [[Splice site|splice sites]] and promoter sequences
|
* Normal
|
* Normal to ↑(Increased)
|
* Normal
|
* Normal
|
* (Increased)
|Normal to increased
|
* Target cells
* Anisopoikilocytosis
|-
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" |Thromobcytosis
!G6pd deficiency
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Iron deficiency anemia|Iron deficiency anemia]]
|
Inflammatory diseases
* [[Normocytic anemia|Normocytic]]
 
|
[[Splenectomy]]
* + ([[Intravascular]])
 
|
[[Essential thrombocytosis|Essential thrombocytosis]]
* Intrinsic
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↑(Increased) but usually causes resolution within 4-7 days
|
* ↓(Decreased)
|
* [[Back pain]]
* [[Hemoglobinuria]]
|History of using
* Sulfa drugs
* Antimalarials
* infections,
* Fava Beans
|
* [[Back pain]]
|
* Defect in [[Glucose-6-phosphate dehydrogenase|G6PD]] enzyme
* X-Linked [[recessive]]
|
* ↓(Decreased)
|
* Normal to ↑(Increased)
|
* Normal
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↑(Increased)
|
|
* Digital pain
* RBC with [[Heinz bodies]]
* [[Gangrene]]
* Bite cells
* [[Erythromelalgia]]
* Blister cells
* Headache
*  
* [[Paresthesia|Paresthesias]] 
* [[Transient ischemic attack|Transient ischemic attacks]]
|−
|−
|−
| +/-
| +/-
|↑
|Ν/↑
|N
| -
|-
|-
| rowspan="5" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Qualitative Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
!Pyruvate Kinase deficiency
| rowspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
|
| align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Glanzmann's thrombasthenia|Glanzmann’s thrombasthenia]]
* [[Normocytic anemia|Normocytic]]
|
* + ([[Intravascular]])
|
* Intrinsic
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* [[Hydrops fetalis]]
* [[Neonatal hyperbilirubinemia]]
* [[Iron overload]]
* [[Perinatal]] complications
|
* [[Gallstones]]
|
* Skin ulcers
* Splenomegaly of varying degree
|
*  Mutation in the ''[[PKLR]]'' and PKM gene mutations
* [[Autosomal recessive]]
|
* Normal
|
* ↑(Increased)
|
* Normal
|
* Normal
|
|
* Positive family history
* ↑(Increased)
|<nowiki>+</nowiki>
|<nowiki>--</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
|Rare
|N/↓
|↑
|N
|
|
* AR inheritance
* Prickle cells
* Absence of the platelet Gp IIb/IIIa receptor/
* Polychromatophilic erythrocytes
* Diminished for GP 2B-3A on [[Flow cytometry|f<abbr>low cytometry</abbr>]]
|-
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Bernard-Soulier syndrome]]<ref name="pmid30077511">{{cite journal |vauthors=Dupuis A, Gachet C |title=Inherited platelet disorders : Management of the bleeding risk |journal=Transfus Clin Biol |volume=25 |issue=3 |pages=228–235 |date=September 2018 |pmid=30077511 |doi=10.1016/j.tracli.2018.07.003 |url=}}</ref><ref name="pmid29227167">{{cite journal |vauthors=Andres O, Henning K, Strauß G, Pflug A, Manukjan G, Schulze H |title=Diagnosis of platelet function disorders: A standardized, rational, and modular flow cytometric approach |journal=Platelets |volume=29 |issue=4 |pages=347–356 |date=June 2018 |pmid=29227167 |doi=10.1080/09537104.2017.1386297 |url=}}</ref>
!Sickle cell anemia
|
* [[Normocytic anemia|Normocytic]]
|
* + ([[Intravascular]])
|
* Intrinsic
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Painful crisis
** [[Dactylitis]]
** [[Priapism]]
** [[Acute chest syndrome]]
** [[Avascular necrosis|Avascular Necrosis]]
** [[Stroke]]
* [[Autosplenectomy]]
* [[Salmonella|Salmonella osteomyelitis]]
*
|
* [[High altitude]]
* Low [[Oxygen]]
* [[Acidosis]]
* African-American race
* [[Parvovirus B19]] infection
|
* [[Dactylitis]]
* [[Priapism]]
|
* Hbs [[point mutation]] causes a single [[Amino acid|amino]]  [[Amino acid|acid]] replacement in β chain
|Normal or moderately elevated
|
* Normal
|
* Normal
|Normal or moderately elevated
|
|
* Positive family history
* ↓(Decreased)
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
| -
|N/
|↑
|N
|N
|N
|
|
* AR inheritance
* Normal
* Absence of the platelet Gp Ib-IX-V receptor
|
* On PBS: giant platelets
* Increased [[erythropoiesis]]
* Ristocetin - no aggregation
* [[Howell-Jolly bodies]]
* [[Anisocytosis]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" |[[Platelet storage pool deficiency|Platelet storage pool disorder (SPD)]]:
!HbC disease
*[[Hermansky-Pudlak syndrome]]
|
*[[Chediak-Higashi syndrome]]
* [[Normocytic anemia|Normocytic]]
*[[Gray platelet syndrome]]
|
* + ([[Intravascular]])
|
* Intrinsic
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* [[Splenomegaly]]
* [[Gallstone disease|Cholelithiasis]]
* [[Avascular necrosis|Avascular necrosis of the femoral head]]
* [[Joint pains]]
* Increased risk of [[Infection|infections]]
|
|
* Positive family history
* Gallstones
*Hairy-cell leukemia
* Cardiovascular bypass
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
| -
|N/↓
|↑
|N
|N
|N
|
|
* AD inheritance
* Enlarged spleen
* Abnormalities of platelet granule formation
|
* Glutamic acid–to-lysine mutation in
β-globin
|
* Normal
|
* Normal
|
* Normal
|
* Normal
|
* ↓(Decreased)
|<nowiki>--</nowiki>
|
*Hemoglobin  Crystals inside RBCs
*Target cells
|-
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" |Acquired Disorders of [[Platelet]] Function<span name="harr_c115s002s004s002p001"></span><span name="9100808"></span>
!Paroxysmal nocturnal hemoglobinuria(PNH)<ref name="pmid1402472">{{cite journal |vauthors=Bunyaratvej A, Butthep P |title=Cytometric analysis of paroxysmal nocturnal hemoglobinuria erythrocytes |journal=J Med Assoc Thai |volume=75 Suppl 1 |issue= |pages=237–42 |date=January 1992 |pmid=1402472 |doi= |url=}}</ref><ref name="pmid25553278">{{cite journal |vauthors=Kahng J, Kim Y, Kim JO, Koh K, Lee JW, Han K |title=A novel marker for screening paroxysmal nocturnal hemoglobinuria using routine complete blood count and cell population data |journal=Ann Lab Med |volume=35 |issue=1 |pages=35–40 |date=January 2015 |pmid=25553278 |pmc=4272963 |doi=10.3343/alm.2015.35.1.35 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC;" |
|
* [[Chronic renal failure pathophysiology|Uremia]]
* [[Normocytic anemia|Normocytic]]
* Cardiopulmonary bypass
|
* Hematologic disorders such as: [[Myeloproliferative disease|myeloproliferative]] and [[Myelodysplastic syndrome|myelodysplastic syndromes]]
* + ([[Intravascular]])
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* Intrinsic
|<nowiki>+</nowiki>
|
| +/-
* [[Normochromic anemia|Normochromic]]
| +/-
|
|N/↓
* ↑(Increased)
|↑
|N
|N
|N
|<nowiki>-</nowiki>
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Von Willebrand disease|Von Willebrand Disease]]<span name="harr_c115s002s005p001"></span><span name="9100810"></span> <ref name="pmid25196510">{{cite journal |vauthors=Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD |title=Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project |journal=Haemophilia |volume=20 |issue=6 |pages=831–5 |date=November 2014 |pmid=25196510 |pmc=4251588 |doi=10.1111/hae.12503 |url=}}</ref><ref name="pmid25196510">{{cite journal |vauthors=Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD |title=Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project |journal=Haemophilia |volume=20 |issue=6 |pages=831–5 |date=November 2014 |pmid=25196510 |pmc=4251588 |doi=10.1111/hae.12503 |url=}}</ref><ref name="pmid16985174">{{cite journal |vauthors=Goodeve A, Eikenboom J, Castaman G, Rodeghiero F, Federici AB, Batlle J, Meyer D, Mazurier C, Goudemand J, Schneppenheim R, Budde U, Ingerslev J, Habart D, Vorlova Z, Holmberg L, Lethagen S, Pasi J, Hill F, Hashemi Soteh M, Baronciani L, Hallden C, Guilliatt A, Lester W, Peake I |title=Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD) |journal=Blood |volume=109 |issue=1 |pages=112–21 |date=January 2007 |pmid=16985174 |doi=10.1182/blood-2006-05-020784 |url=}}</ref><ref name="pmid9579642">{{cite journal |vauthors=Mammen EF, Comp PC, Gosselin R, Greenberg C, Hoots WK, Kessler CM, Larkin EC, Liles D, Nugent DJ |title=PFA-100 system: a new method for assessment of platelet dysfunction |journal=Semin. Thromb. Hemost. |volume=24 |issue=2 |pages=195–202 |date=1998 |pmid=9579642 |doi=10.1055/s-2007-995840 |url=}}</ref><ref name="pmid258585642">{{cite journal |vauthors=Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J |title=Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=13 |issue=7 |pages=1345–50 |date=July 2015 |pmid=25858564 |pmc=5576173 |doi=10.1111/jth.12964 |url=}}</ref>
 
|
|
* Easy bruising
* ↑(Increased)
* [[Epistaxis]]
* Oral cavity bleeding
* Bleeding after dental extraction/surgery
* [[Menorrhagia]]
* [[Postpartum hemorrhage]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| +/-
| +/-
|
|↑
|↑
|N
|See the table below for the details about  types.
|-
| rowspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Vessel wall disorders
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Metabolism|Metabolic]] and [[Inflammation|Inflammatory]] Disorders
| align="left" style="padding: 5px 5px; background: #DCDCDC;" |
* Acute febrile illnesses
* [[Cryoglobulinemia|Mixed cryoglobulinemia]]
* [[Monoclonal gammopathy|Monoclonal gammopathies]]
* Certain pathogens, such as the rickettsiae causing [[Rocky Mountain spotted fever]]
* [[Vitamin C]] deficiency
* [[Cushing's syndrome|Cushing’s syndrome]]
* Chronic [[glucocorticoid]] therapy
* [[Ageing|Aging]] 
* [[Vasculitis]] such as Henoch-Schönlein,
|
|
* History of the underlying disease.
* (Decreased)
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| -
| -
|N
|↑/N
|N
|N
|N
|<nowiki>-</nowiki>
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited Disorders of the [[Vessel wall|Vessel Wall]]
| align="left" style="padding: 5px 5px; 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]])
|
|
* Positive family history
* [[Hemolysis|Chronic hemolysis]]
|<nowiki>+</nowiki>
* [[Fatigue]]
|<nowiki>+</nowiki>
* [[Chest pain]]
|<nowiki>+/-</nowiki>
* [[Dyspnea]] on exertion
| -
* [[Headache]]
| -
|N
|↑/N
|N
|N
|N
|<nowiki>-</nowiki>
|-
| rowspan="12" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Coagulation factor disorders
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Fibrinogen]] deficiency
| 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]]
* [[Fibrinogen#Congenital hypodysfibrinogenemia|Congenital hypodysfibrinogenemia]][[Fibrinogen#Cryofibrinogenemia|Cryofibrinogenemia]]
* [[Fibrinogen#Acquired hypofibrinogenemia|Acquired hypofibrinogenemia]]
|
|
* [[Epistaxis]]
* Associated with [[aplastic anemia]]
* Easy [[Bruise|bruising]]
* [[Menorrhagia]]
* [[Muscle]] bleeds
* [[Hemarthrosis]]
* [[Bleeding]] from the [[umbilical cord]] stump after birth
* Bleeding after [[dental surgery]] or tooth extraction
* Abnormal bleeding during or after injury, surgery, or childbirth
 
* [[Gastrointestinal tract|Gastrointestinal]] [[hemorrhage]]
* [[Cerebral hemorrhage]]
* [[Thrombosis]]
* [[Thrombosis]]
|_
| +
| +
| +/-
| +
|N
|↑
|↑
|↑
|↑
|
|
* Impaired fibrin cross linking or clot dissolution.
* [[Hepatomegaly]]
* [[Ascites]]
* [[Papilledema]]
* Skin nodules
|
* [[PIGA]] gene mutations
* Impaired synthesis of [[GPI anchor]] for decay-accelerating  factor [DAF/[[CD55]]] and membrane inhibitor  of reactive lysis [MIRL/[[CD59]]]


* The severity of bleeding in patients with fibrinogen disorders can be mild or severe, with higher bleeding risk in those with afibrinogenemia or lower levels of functional fibrinogen.  The age of onset is also variable, with earlier onset in those with more severe deficiency.
|
* Normal
|
* ↓(Decreased)
|
* Normal
|
* ↑(Increased)
|
* ↓(Decreased)
|<nowiki>--</nowiki>
|<nowiki>--</nowiki>
|-
|-
| colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Prothrombin deficiency]]
!Hereditary spherocytosis
|
|
* Easy [[bruising]]
* [[Normocytic anemia|Normocytic]]
* [[Epistaxis]]
|
* Soft-tissue hemorrhage
* + ([[Intravascular]])
* Excessive postoperative bleeding
|
* [[Menorrhagia]]
* Intrinsic
* Muscle [[Hematoma|hematomas]]
|
* [[Hemarthrosis]]
* [[Normochromic anemia|Normochromic]]
* [[Intracranial hemorrhage|Intracranial]] bleeding
|
|<nowiki>+</nowiki>
* ↑(Increased)
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* ↑(Increased)
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* ↓(Decreased)
|N
|
|N
* [[Splenomegaly]]  
|
* [[Aplastic crisis]]
|
|
|↑
* Associated with [[parvovirus B19]]
|<nowiki>-</nowiki>
* [[Cholelithiasis]]
* Megaloblastic crisis
|
* [[Splenomegaly]]
|
* Mutations in [[Ankyrin]],[[Band 3]], [[Protein 4.2]] and [[spectrin]]
|
* Normal
|
* ↓(Decreased)
|
* Normal
|
* (Increased)
|
* Normal
|<nowiki>--</nowiki>
|
* Small, round [[Red blood cell|RBC]]<nowiki/>s with less surface
area and no central pallor ( MCHC)
|-
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor V deficiency]]
!Microangiopathic hemolytic anemia<ref name="pmid26251142">{{cite journal |vauthors=Morishita E |title=[Diagnosis and treatment of microangiopathic hemolytic anemia] |language=Japanese |journal=Rinsho Ketsueki |volume=56 |issue=7 |pages=795–806 |date=July 2015 |pmid=26251142 |doi=10.11406/rinketsu.56.795 |url=}}</ref>
|
|
* Excessive bruising with minor injuries
* [[Normocytic anemia|Normocytic]]
* [[Epistaxis]]
|
* [[Hemarthrosis]]  
* + ([[Intravascular]])
* [[Menorrhagia]]
|
* [[Intracerebral hemorrhage|Intracerebral hemorrhages]]
* Intrinsic
* [[Pulmonary hemorrhage]]
|
|_
* [[Normochromic anemia|Normochromic]]
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* ↑(Increased)
| +
|
| +
* ↑(Increased)
|N
|
|
* ↓(Decreased)
|
|
|
* Purpura
|N
* Confusion
|The severity of bleeding is only partly related to the degree of factor V deficiency. Some patients with undetectable plasma levels of factor V experience only relatively mild bleeding.
* Aphasia
* Diplopia
|Associated with
* DIC
* TTP
* HUS
* SLE
* HELLP
syndrome
* Hypertensive emergency
|
* Numbness of an arm or hand
* Jaundice
* Pale conjunctiva
|<nowiki>--</nowiki>
|
* Normal
|
* ↓(Decreased)
|
* Normal
|<nowiki>--</nowiki>
|
* (Increased)
|<nowiki>--</nowiki>
|
* Helmet cells
|-
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor VII deficiency]]
!Macroangiopathic hemolytic anemia
|
* [[Normocytic anemia|Normocytic]]
|
* + ([[Intravascular]])
|
* Intrinsic
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* [[Pallor]]
* [[Fatigue]]
|Associated with
* Prosthetic heart valves
* Aortic stenosis
|
* Signs of anemia
* Complications of hemolysis
* Decreased vascular volume
|
* Autoimmune
|
|
* Easy [[Bruise|bruising]]
* Normal
* Mucosal bleeding
* Postoperative bleeding
* [[Menorrhagia]]
* Soft tissue hematomas
* [[Thrombosis]]
|
|
* ↓(Decreased)
|
|
|<nowiki>+</nowiki>
* Normal
| +
|<nowiki>--</nowiki>
| +
|<nowiki>--</nowiki>
|N
|<nowiki>--</nowiki>
|
|
|↑
* Spherocytes or schistocytes
|N
|N
|Thrombosis occurs in inherited factor VII deficiency  most cases are associated with the administration of factor VII replacement therapy
|-
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor X deficiency]]
!Infections
|
* [[Normocytic anemia|Normocytic]]
|
* + ([[Intravascular]])
|
* Intrinsic
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* [[Fever]]
|Associated with
* Malaria
* Babesia
|
* Fever
* Signs of shock
* Headache
|<nowiki>--</nowiki>
|
* Normal
|
* Normal
|
|
* Prolonged bleeding following circumcision
* Normal
 
|<nowiki>--</nowiki>
* Easy [[Bruise|bruising]]
|<nowiki>--</nowiki>
* [[Hematuria]]
|<nowiki>--</nowiki>
* [[Menorrhagia]]
|
* Abortion
* Trophozoite
* Postpartum hemorrhage
* Maltese crosses
* Epistaxis
* Pseudotumors
* Intracranial bleeding
* Hemarthroses
|<nowiki>+</nowiki>
| +
| +
| +
| +
|N
|N
|↑
|↑
|N
|<nowiki>-</nowiki>
|-
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor XII|Factor XII deficiency]]
!Iron deficiency anemia(Early)
|
* [[Normocytic anemia|Normocytic]]
| --
| --
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Normal
|
* [[Fatigue]]
* [[Headache]]
|
* Pica
* Glossitis
* Cheilosis
|
* Koilonychia
* Conjuctival pallor
* Dry skin
|<nowiki>--</nowiki>
|
* Normal
|
* ↓(Decreased)
|
* ↑(Increased)
|
* ↑(Increased)
|
|
* Majority,asymptomatic
* ↓(Decreased)
* Recurrent miscarriages
* Painful leg ulcers
|_
|_
|_
|_
|_
|N
|N
|N
|↑
|N
|
|
* ↓(Decreased)
|
* Microcytosis
* Pencil cells
* Ellyptocytosis
* Hypochromasia
|-
|-
| colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[High-molecular-weight kininogen|High molecular weight kininogen (HMWK)]] deficiency
!Anemia of chronic disease
|
|
* Possibility of positive family history of bleeding
* [[Normocytic anemia|Normocytic]]
|_
| --
|_
| --
|_
|_
|_
|N
|N
|N
|
|N
|
|
* [[Normochromic anemia|Normochromic]]
|
* Normal
|Normal or ↓(Decreased)
|
* Normal
|
* [[Fatigue]]
* [[Headache]]
|
* History of:
** Rheumatoid arthritis
** SLE
** Neoplastic disorders
** Chronic kidney disease
|<nowiki>--</nowiki>
|<nowiki>--</nowiki>
|
* ↑(Increased)
|
* ↓(Decreased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Normocytic cells but may become microcytic
|-
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Prekallikrein]] deficiency
!Aplastic anemia
|
* [[Normocytic anemia|Normocytic]]
| --
| --
|
* [[Normochromic anemia|Normochromic]]
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Normal
|
* Symptoms based on underlying condition
|
* Exposure to [[Radiation]]
* Drugs like [[Benzene]],[[chloramphenicol]],[[Alkylating agent|alkylating]] agents
* Viral infections like [[EBV]],[[HIV]],[[Hepatitis]]
* [[Fanconi anemia]]
* Idiopathic like [[Immune]] mediated, primary stem cell defect
|
* Short stature
* [[Cafe-au-lait spots]]
* Thumb defects
* [[Radius (bone)|Radial]] defects
|
* Constitutive expression of Tbet
* Mutations in the perforin gene
* Mutations in ''SAP gene''
|
* Normal
|
* ↓(Decreased)
|
* ↓(Decreased)
|
|
* Possibility of positive family history of bleeding
* Normal
|_
|_
|_
|_
|_
|N
|N
|N
|↑
|N
|
|
* ↑(Increased)
|
* ↓(Decreased)
|
* [[Pancytopenia]]
* Fatty [[Infiltration (medical)|infiltration]]
|-
|-
| colspan="2" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor XIII deficiency]]
!Chronic kidney disease
| style="padding: 5px 5px; background: #DCDCDC;" |Types:
* Sub unit A mutation disease (more common)
* Sub unit B mutation disease
|
|
* Possibility of positive family history of bleeding
* [[Normocytic anemia|Normocytic]]
| -/+
| --
|<nowiki>-/+</nowiki>
| --
|<nowiki>-/+</nowiki>
|<nowiki>-/+</nowiki>
|<nowiki>-/+</nowiki>
|N
|N/
|N/↑
|N
|N
|
|
* Impaired fibrin cross linking or clot dissolution
* [[Normochromic anemia|Normochromic]]
* The severity of factor XIII deficiency bleeds can be different in different patients
|
* ↑(Increased)
|Normal /↑(Increased)
|
* Normal
|
* [[Polyuria]]
* [[Hematuria]]
* [[Edema]]
|
* [[Pericarditis]]
* [[Encephalopathy]]
* [[Incontinence|Rectal incontinence]]
* Decreased [[libido]]
* [[Restless legs syndrome|Restless leg syndrome]]
|
* [[Hypertension]]
* Mostly normal
|<nowiki>--</nowiki>
|
* ↑(Increased)
|
* ↓(Decreased)
|<nowiki>--</nowiki>
|
* ↓(Decreased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Normal
|-
|-
| rowspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemophilia]]<ref name="pmid94489952">{{cite journal |vauthors=Aviña-Zubieta JA, Galindo-Rodriguez G, Lavalle C |title=Rheumatic manifestations of hematologic disorders |journal=Curr Opin Rheumatol |volume=10 |issue=1 |pages=86–90 |date=January 1998 |pmid=9448995 |doi= |url=}}</ref><ref name="pmid16551972">{{cite journal |vauthors=Plug I, Mauser-Bunschoten EP, Bröcker-Vriends AH, van Amstel HK, van der Bom JG, van Diemen-Homan JE, Willemse J, Rosendaal FR |title=Bleeding in carriers of hemophilia |journal=Blood |volume=108 |issue=1 |pages=52–6 |date=July 2006 |pmid=16551972 |doi=10.1182/blood-2005-09-3879 |url=}}</ref><ref name="pmid25059285">{{cite journal |vauthors=Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A |title=Definitions in hemophilia: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=12 |issue=11 |pages=1935–9 |date=November 2014 |pmid=25059285 |doi=10.1111/jth.12672 |url=}}</ref><ref name="pmid11307831">{{cite journal |vauthors=White GC, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J |title=Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis |journal=Thromb. Haemost. |volume=85 |issue=3 |pages=560 |date=March 2001 |pmid=11307831 |doi= |url=}}</ref><ref name="pmid24026910">{{cite journal |vauthors=Favaloro EJ, Meijer P, Jennings I, Sioufi J, Bonar RA, Kitchen DP, Kershaw G, Lippi G |title=Problems and solutions in laboratory testing for hemophilia |journal=Semin. Thromb. Hemost. |volume=39 |issue=7 |pages=816–33 |date=October 2013 |pmid=24026910 |doi=10.1055/s-0033-1356573 |url=}}</ref><ref name="pmid250592852">{{cite journal |vauthors=Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A |title=Definitions in hemophilia: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=12 |issue=11 |pages=1935–9 |date=November 2014 |pmid=25059285 |doi=10.1111/jth.12672 |url=}}</ref>
!Folate deficiency
 
|
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type A deficiency
* [[Macrocytic anemia|Macrocytic]]
| --
| --
|
* Anisochromic
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Normal
|
* No neurological symptoms vs [[Vitamin B12 deficiency|B12 deficiency]]
* [[Odinophagia|Odynophagia]]
* [[Stomatitis|Angular stomatitis]]
|
* Alcoholics
* History of using drugs like [[methotrexate]], [[trimethoprim]] and [[phenytoin]]
* Low socioeconomic groups with poor nutrition
* Older people
* Pregnant and lactating women
|
* [[Glossitis]]
* Signs of heart failure
* [[Anencephaly]] and [[spina bifida]]
|
* Impaired [[DNA]] synthesis
|
* Normal
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* ↑(Increased)
|
* ↑(Increased)
|
|
* Eeasy [[Bruise|bruising]]
* [[Macrocytosis|RBC macrocytosis]]
* Inadequate clotting in [[trauma]] or mild injury
* [[Neutrophil|Hypersegmented neutrophils]]
* Spontaneous hemorrhage
* [[Pancytopenia]] in severe cases
* [[Hemarthrosis]]
*  
* [[Epistaxis]]
* [[Gingival bleeding]]
|_
|_
| +
| +
|<nowiki>+</nowiki>
|N
|N
|N
|↑
|N
|<nowiki>-</nowiki>
|-
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type B deficiency
!Vitamin B12 deficiency
|
* [[Macrocytic anemia|Macrocytic]]
| --
| --
|
* Anisochromic
|
* ↑(Increased)
|
* ↓(Decreased)
|
* Normal
|
* [[Psychosis]]
* [[Insomnia]]
* [[Depression]]
* Cognitive slowing
* [[Restless leg syndrome]]
|
* [[Pernicious anemia]]
* [[Crohn's disease]]
* [[Gastrectomy]]
* [[Vegan|Veganism]]
* [[Diphyllobothrium|Diphyllobothrium latum]] infection
|
* Neurological deficit
* [[Myelopathy]]
* [[Memory loss]] with reduced attention span
* [[Nystagmus]]
* Positive [[Romberg test|romberg sign]]
* Positive [[Lhermitte's sign]]
|
* Impaired DNA synthesis
|
* Normal
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* ↑(Increased)
|
|
* Neonatal bleeding
* ↑(Increased)
* Trauma-related soft-tissue hemorrhage
|
* [[Hemarthrosis]]
* [[Senile]] [[neutrophil]]
* [[Hematoma|Hematomas]]  
* [[Anisocytosis]]  
|_
* [[Ovalocytosis|Ovalocytes]]
|_
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|N
|N
|N
|↑
|N
|<nowiki>-</nowiki>
|-
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type C deficiency
!Orotic aciduria
|
* [[Macrocytic anemia|Macrocytic]]
| --
| --
|
* Anisochromic
|
|
* Family history
* ↑(Increased)
* Bleeding after surgery or injury
|
|_
* ↓(Decreased)
|_
|
|<nowiki>+</nowiki>
* Normal
|Rare
|
|Rare
* Coma
|N
* Gastro-intestinal manifestation
|N
|
|N
* Episodic vomiting
|↑
* [[Rhabdomyolysis]]
|N
|
| -
* Neurological manifestation
|
* [[Autosomal recessive]]
* Deficiency of enzyme UMPS
|
* Normal
|
* ↑(Increased)
|
* ↑(Increased)
|
* ↓(Decreased)
|
* (Increased)
|
* ↑(Increased)
| --
|-
|-
| rowspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Rare diseases
!Fanconi anemia
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Disseminated intravascular coagulation|Disseminated Intravascular Coagulation]]
|
| style="padding: 5px 5px; background: #DCDCDC;" |
* [[Macrocytic anemia|Macrocytic]]
* [[Trauma]]
| --
* Burn
| --
* [[Crush injury]]
|
* [[Sepsis]]
* Anisochromic
* [[Malignancy]]
|
* Obstetric complication: abruption, amniotic fluid embolism
* ↑(Increased)
* [[Hemolytic anemia]]
|
|<nowiki>+</nowiki>
* ↓(Decreased)
|<nowiki>+</nowiki>
|
|_
* Normal
|<nowiki>+</nowiki>
|
|<nowiki>+</nowiki>
* [[Hypopigmentation]]
|
* [[Café au lait spot|Cafe-au-lait patches]]
|↑
* Radial ray abnormality
|↑
|
|
* History of [[anemia]] at age 16
|N
|
| -
* Significant for B\L short thumbs
|
* [[Autosomal recessive]]
* [[X-linked recessive]]
|
* Normal
|
* ↑(Increased)
|
* ↑(Increased)
|
* (Decreased)
|
* (Increased)
|
* (Increased)
|
* Normal appearing WBC, RBC and Platelets
* But the number is greatly reduced
|-
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Vitamin K Deficiency]]
!Diamond-Blackfan anemia
| style="padding: 5px 5px; background: #DCDCDC;" |
|
* Bleeding after trauma
* [[Macrocytic anemia|Macrocytic]]
* [[Epistaxis]]
| --
* [[Hematoma]]  
| --
* Gastrointestinal bleeding
|
* [[Menorrhagia]]  
* Anisochromic
* [[Hematuria]]
|<nowiki>--</nowiki>
* Gum bleeding
|
* Oozing from venipuncture sites
* ↓(Decreased)
* Easy [[Bruise|bruisability]]
|
|<nowiki>+</nowiki>
* Normal
| +
|
| +
* [[Pale skin]]
|<nowiki>+</nowiki>
* Sleepiness
|<nowiki>+</nowiki>
* [[Murmur|Heart murmurs]]
|N
|
|
* Associated with [[myelodysplastic syndrome]]
|↑
* Increased risk of [[AML]]
|Normal or mildly prolonged
|
|N
*Triphalangeal  thumbs
| -
*[[Short stature]]
|}
* [[Microcephaly]]
 
* [[Hypertelorism]]
{| class="wikitable"
* [[Ptosis]]
|+
* [[Micrognathia]]
Different types of Von-Willebrand diseases can be differentiated from each other based on the following table:<ref name="pmid258585643">{{cite journal |vauthors=Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J |title=Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=13 |issue=7 |pages=1345–50 |date=July 2015 |pmid=25858564 |pmc=5576173 |doi=10.1111/jth.12964 |url=}}</ref>
|Mutations in:
!Type of VWD
* RPL5
!
* RPL11
!Type of factor deficiency
* RPL35A
!Prevalence
* RPS7
!Inheritance pattern
* RPS10
!Clinical manifestations
* RPS17
!VWF activity
* RPS19
!RIPA
* RPS24
!Factor VIII
* RPS26
|
* Normal
|
* (Increased)
|
* (Increased)
|
* ↓(Decreased)
|
* ↑(Increased)
|
* ↑(Increased)
|<nowiki>--</nowiki>
|-
|-
|Type 1
!Liver disease
|
|
|Quantitative/ partial
* [[Macrocytic anemia|Macrocytic]]
|60-70%
| --
|AD
| --
|
|
* Bleeding severity mild to severe
* Anisochromic
|↓
|
|
* ↑(Increased)
|
|
* ↑(Increased)
|
* Normal
|
* [[Jaundice]]
* [[Abdominal pain]]
* [[Itchy skin]]
|
* [[Hepatitis]]
* Binge drinking
* Gall bladder disease
|
* [[Ascites]]
* Right upper quadrant pain
* [[Hepatomegaly]]
* Swelling in the legs
* [[Ankle swelling]]
|<nowiki>--</nowiki>
|
* Normal
|
* ↑(Increased)
|
* ↑(Increased)
|
* (Decreased)
|
* ↑(Increased)
|
* ↑(Increased)
|
* [[Macrocyte|Round macrocytes]]
* [[Macrocyte|Target macrocytes]]
|-
|-
| rowspan="4" |Type 2
!Alcoholism
|2A<ref name="pmid1537829">{{cite journal |vauthors=Lyons SE, Bruck ME, Bowie EJ, Ginsburg D |title=Impaired intracellular transport produced by a subset of type IIA von Willebrand disease mutations |journal=J. Biol. Chem. |volume=267 |issue=7 |pages=4424–30 |date=March 1992 |pmid=1537829 |doi= |url=}}</ref>
|Qualitative
|10%
|AD/AR
|
|
* Moderate to severe bleeding
* [[Macrocytic anemia|Macrocytic]]
|
| --
|
| --
|N or ↓
|
|-
* Anisochromic
|2B
|
|Qualitative
* ↑(Increased)
|5%
|
|AD
* ↑(Increased)
|
* Normal
|
* [[Memory impairment]]
* [[Nausea]]
* [[Sweating]]
|
* Increased history of alcohol intake
* [[Folic acid deficiency]]
|
* Truncal obesity
* [[Asterixis]]
* [[Encephalopathy]]
* [[Spider angiomas]]
* [[Hematemesis]]
* [[Gynecomastia]]
*
|<nowiki>--</nowiki>
|
* Normal
|
|
* [[Thrombocytopenia]]
* ↑(Increased)
* Moderate to severe bleeding
|↓
|
|N or↓
|-
|2M
|Qualitative
|<1%
|AD/AR
|
|
* Moderate to severe bleeding
* ↑(Increased)
|↓
|↓
|N or ↓
|-
|2N
|Qualitative
|<1%
|AR
|
|
* Clinically similar to [[hemophilia A]] with joint, soft tissue, urinary bleeding
* ↓(Decreased)
|N
|N
|
|-
|Type 3
|
|
|Complete deficiency
* ↑(Increased)
|1-2%
|AR
|
|
* Clinically similar to [[hemophilia A]] with joint and soft tissue bleeding
* ↑(Increased)
* Severe [[mucosal bleeding]]
|Absent
|↓
|
|
Low, 1-10%
* [[Macrocyte|Oval macrocytes]]
* [[Neutrophil|Hypersegmented neutrophils]]
|}
|}
For more information on Von Willebrand disease [[ von willebrand disease | click here]]


{| class="wikitable"
{| class="wikitable"

Latest revision as of 20:56, 26 February 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S; Shyam Patel [2], Irfan Dotani [3]

Overview

A variety of conditions comprise the differential diagnosis of autoimmune hemolytic anemia. These include microangiopathic hemolytic anemia, paroxysmal cold hemoglobinuria, paroxysmal nocturnal hemoglobinuria, hereditary spherocytosis, pernicious anemia, and chronic lymphocytic leukemia. The diagnosis of autoimmune hemolytic anemia can sometimes be made by first ruling out these other causes. It is important to distinguish amongst these conditions since the prognosis and treatment of each condition is different.

Differentiating Autoimmune hemolytic anemia from other Diseases

Type of anemia MCV Hemolysis Intrinsic/Extrinsic Hb concentration RDW Reticulocytosis Haptoglobin levels Specific Symptoms Specific History Physical examination Genetics Iron studies Specific finding on blood smear
Hepcidin Serum iron Serum Tfr level Transferrin or TIBC Ferritin % Transferrin saturation
Autoimmune hemolytic anemia
  • Extrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
Associated with:
  • SLE
  • CLL
  • Mycoplasia pneumoniae infection
  • Painful, blue fingers and toes with cold weather
--
  • Normal
  • ↓(Decreased)
  • Normal
-- -- --
  • RBC agglutination
Iron deficiency anemia -- --
  • ↑(Increased)
  • Normal or ↓(Decreased)
  • Normal
--
  • Normal
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↓↓↓(Decreased)
Lead poisoning -- --
  • Normal
  • Normal or ↓(Decreased)
  • Normal
  • House painted with chipped paint
--
  • Normal
  • Normal or ↓(Decreased)
  • Normal
  • Normal
  • Normal or ↓(Decreased)
--
  • RBCs retain aggregates of rRNA (basophilic stippling)
Sideroblastic anemia -- --
  • Normal
  • Normal or ↓(Decreased)
  • Normal
  • Normal
  • ↑(Increased)
  • Normal
  • Normal or ↓(Decreased)
  • ↑(Increased)
--
Anemia of chronic disease -- --
  • Normal
  • Normal or ↓(Decreased)
  • Normal
-- --
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
  • ↓(Decreased)
  • ↑(Increased)
-- --
Thalassemia -- --
  • Normal
  • Thalassemia trait

Normal or ↓(Decreased)

  • Thalassemia Syndromes

↑(Increased)

  • Normal
α-thalassemia

β-thalassemia

α-thalassemia
  • α- globin gene deletions
  • Cis deletions
  • Trans deletions

β-thalassemia

  • Normal
  • Normal to ↑(Increased)
  • Normal
  • Normal
  • ↑(Increased)
Normal to increased
  • Target cells
  • Anisopoikilocytosis
G6pd deficiency
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased) but usually causes resolution within 4-7 days
  • ↓(Decreased)
History of using
  • Sulfa drugs
  • Antimalarials
  • infections,
  • Fava Beans
  • ↓(Decreased)
  • Normal to ↑(Increased)
  • Normal
  • ↑(Increased)
  • ↑(Increased)
  • ↑(Increased)
Pyruvate Kinase deficiency
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • Skin ulcers
  • Splenomegaly of varying degree
  • Normal
  • ↑(Increased)
  • Normal
  • Normal
  • ↑(Increased)
--
  • Prickle cells
  • Polychromatophilic erythrocytes
Sickle cell anemia
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
Normal or moderately elevated
  • Normal
  • Normal
Normal or moderately elevated
  • ↓(Decreased)
  • Normal
HbC disease
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • Gallstones
  • Enlarged spleen
  • Glutamic acid–to-lysine mutation in

β-globin

  • Normal
  • Normal
  • Normal
  • Normal
  • ↓(Decreased)
--
  • Hemoglobin Crystals inside RBCs
  • Target cells
Paroxysmal nocturnal hemoglobinuria(PNH)[1][2]
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • PIGA gene mutations
  • Impaired synthesis of GPI anchor for decay-accelerating factor [DAF/CD55] and membrane inhibitor of reactive lysis [MIRL/CD59]
  • Normal
  • ↓(Decreased)
  • Normal
  • ↑(Increased)
  • ↓(Decreased)
-- --
Hereditary spherocytosis
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
  • ↓(Decreased)
  • Normal
  • ↑(Increased)
  • Normal
--
  • Small, round RBCs with less surface

area and no central pallor ( MCHC)

Microangiopathic hemolytic anemia[3]
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • Purpura
  • Confusion
  • Aphasia
  • Diplopia
Associated with
  • DIC
  • TTP
  • HUS
  • SLE
  • HELLP

syndrome

  • Hypertensive emergency
  • Numbness of an arm or hand
  • Jaundice
  • Pale conjunctiva
--
  • Normal
  • ↓(Decreased)
  • Normal
--
  • ↑(Increased)
--
  • Helmet cells
Macroangiopathic hemolytic anemia
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
Associated with
  • Prosthetic heart valves
  • Aortic stenosis
  • Signs of anemia
  • Complications of hemolysis
  • Decreased vascular volume
  • Autoimmune
  • Normal
  • ↓(Decreased)
  • Normal
-- -- --
  • Spherocytes or schistocytes
Infections
  • Intrinsic
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
Associated with
  • Malaria
  • Babesia
  • Fever
  • Signs of shock
  • Headache
--
  • Normal
  • Normal
  • Normal
-- -- --
  • Trophozoite
  • Maltese crosses
Iron deficiency anemia(Early) -- --
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
  • Pica
  • Glossitis
  • Cheilosis
  • Koilonychia
  • Conjuctival pallor
  • Dry skin
--
  • Normal
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↓(Decreased)
  • Microcytosis
  • Pencil cells
  • Ellyptocytosis
  • Hypochromasia
Anemia of chronic disease -- --
  • Normal
Normal or ↓(Decreased)
  • Normal
  • History of:
    • Rheumatoid arthritis
    • SLE
    • Neoplastic disorders
    • Chronic kidney disease
-- --
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↓(Decreased)
  • Normocytic cells but may become microcytic
Aplastic anemia -- --
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
  • Symptoms based on underlying condition
  • Constitutive expression of Tbet
  • Mutations in the perforin gene
  • Mutations in SAP gene
  • Normal
  • ↓(Decreased)
  • ↓(Decreased)
  • Normal
  • ↑(Increased)
  • ↓(Decreased)
Chronic kidney disease -- --
  • ↑(Increased)
Normal /↑(Increased)
  • Normal
--
  • ↑(Increased)
  • ↓(Decreased)
--
  • ↓(Decreased)
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
Folate deficiency -- --
  • Anisochromic
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
  • Alcoholics
  • History of using drugs like methotrexate, trimethoprim and phenytoin
  • Low socioeconomic groups with poor nutrition
  • Older people
  • Pregnant and lactating women
  • Impaired DNA synthesis
  • Normal
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
Vitamin B12 deficiency -- --
  • Anisochromic
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
  • Impaired DNA synthesis
  • Normal
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
Orotic aciduria -- --
  • Anisochromic
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
  • Coma
  • Gastro-intestinal manifestation
  • Neurological manifestation
  • Normal
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
--
Fanconi anemia -- --
  • Anisochromic
  • ↑(Increased)
  • ↓(Decreased)
  • Normal
  • Significant for B\L short thumbs
  • Normal
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
  • Normal appearing WBC, RBC and Platelets
  • But the number is greatly reduced
Diamond-Blackfan anemia -- --
  • Anisochromic
--
  • ↓(Decreased)
  • Normal
Mutations in:
  • RPL5
  • RPL11
  • RPL35A
  • RPS7
  • RPS10
  • RPS17
  • RPS19
  • RPS24
  • RPS26
  • Normal
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
--
Liver disease -- --
  • Anisochromic
  • ↑(Increased)
  • ↑(Increased)
  • Normal
  • Hepatitis
  • Binge drinking
  • Gall bladder disease
--
  • Normal
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
Alcoholism -- --
  • Anisochromic
  • ↑(Increased)
  • ↑(Increased)
  • Normal
--
  • Normal
  • ↑(Increased)
  • ↑(Increased)
  • ↓(Decreased)
  • ↑(Increased)
  • ↑(Increased)
Characteristic Causes Pathophysiology Laboratory abnormalities Physical examination Therapy Other associations
Autoimmune hemolytic anemia
  • Polyclonal antibody production that binds to and targets red blood cells for destruction intravascurly or extravascularly[4]
  • Removal of offending agent
  • Corticosteroids
  • Cyclophosphamide
  • Cyclosporine A
  • Azathioprine
  • Rituximab
  • Splenectomy
  • Hemolysis can occur at warm or cold temperatures
Microangiopathic hemolytic anemia
  • Formation of small vessel microthombi
  • Bleeding
  • Thrombosis
  • Pyrexia
  • Altered mental status
  • Neurologic deficits
  • Impaired urine output
  • Can be life-threatening pending the underlying cause
  • TTP required immediate treatment
Paroxysmal cold hemoglobinuria
  • Biphasic hemolysin (IgG) that binds red blood cells and low temperatures and triggers complement-mediated intravascular hemolysis at warm temperatures[5]
  • Associated with syphilis[5]
  • Maternal IgG can cross the placenta and affect the fetus[5]
Paroxysmal nocturnal hemoglobinuria
  • Genetic defect in anchoring proteins for complement factors on red blood cells
  • Hemolysis due to loss of complement inhibition on the red blood cell surface, which in turn is due to defect in CD55 (decay accelerating factor) and CD59 (membrane inhibitor of reactive lysis) or other glycophosphatidylinositol-anchored proteins on the red blood cell membrane
  • Absence of CD55 and CD59 by flow cytometry
  • Splenomegaly
  • Abdominal tenderness
  • Pallor
Hereditary spherocytosis[6]
  • Mutation in ankyrin[6]
  • Mutation in alpha- or beta-spectrin[6]
  • Mutation in band 3[6]
  • Mutation in protein 4.2[6]
  • Positive eosin-5-maleimide binding to red blood cells[6]
  • Positive osmotic fragility testing[6]
  • Spherocytes on peripheral blood smear
  • Can be autosomal dominant or recessive
Pernicious anemia[7]
  • Autoimmune gastritis[7]
  • Production of anti-intrinsic factor antibodies[7]
  • Production of anti-parietal cell antibodies[7]
  • Impaired vitamin B12 absorption due to absence of intrinsic factor
  • Low vitamin B12 level
  • Presence of anti-intrinsic factor antibodies
  • Presence of anti-parietal cell antibodies
  • Associated with diabetes, thyroid disease, vitiligo and other autoimmune conditions
Chronic lymphocytic leukemia[8]
  • Mutations in hematopoietic stem cells and B lymphocytes
  • Clonal proliferation of malignant B lymphocytes
  • Elevated absolute lymphocyte count
  • Anemia (Rai stage III) and thrombocytopenia (Rai stage IV)
  • Chemotherapy with rituximab
  • Ibrutinib
  • Venetoclax
  • Secondary autoimmune hemolytic anemia occurs in 10-25% of patients with CLL
  • Treatment with corticosteroids or anti-leukemic therapy will correct the underlying anemia

References

  1. Bunyaratvej A, Butthep P (January 1992). "Cytometric analysis of paroxysmal nocturnal hemoglobinuria erythrocytes". J Med Assoc Thai. 75 Suppl 1: 237–42. PMID 1402472.
  2. Kahng J, Kim Y, Kim JO, Koh K, Lee JW, Han K (January 2015). "A novel marker for screening paroxysmal nocturnal hemoglobinuria using routine complete blood count and cell population data". Ann Lab Med. 35 (1): 35–40. doi:10.3343/alm.2015.35.1.35. PMC 4272963. PMID 25553278.
  3. Morishita E (July 2015). "[Diagnosis and treatment of microangiopathic hemolytic anemia]". Rinsho Ketsueki (in Japanese). 56 (7): 795–806. doi:10.11406/rinketsu.56.795. PMID 26251142.
  4. 4.0 4.1 4.2 Berentsen S, Sundic T (2015). "Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy". Biomed Res Int. 2015: 363278. doi:10.1155/2015/363278. PMC 4326213. PMID 25705656.
  5. 5.0 5.1 5.2 5.3 Akpoguma AO, Carlisle TL, Lentz SR (2015). "Case report: paroxysmal cold hemoglobinuria presenting during pregnancy". BMC Hematol. 15: 3. doi:10.1186/s12878-015-0023-7. PMC 4334594. PMID 25699184.
  6. 6.0 6.1 6.2 6.3 6.4 6.5 6.6 6.7 Gallagher PG (2013). "Abnormalities of the erythrocyte membrane". Pediatr Clin North Am. 60 (6): 1349–62. doi:10.1016/j.pcl.2013.09.001. PMC 4155395. PMID 24237975.
  7. 7.0 7.1 7.2 7.3 7.4 Chan CQ, Low LL, Lee KH (2016). "Oral Vitamin B12 Replacement for the Treatment of Pernicious Anemia". Front Med (Lausanne). 3: 38. doi:10.3389/fmed.2016.00038. PMC 4993789. PMID 27602354.
  8. Kipps TJ, Stevenson FK, Wu CJ, Croce CM, Packham G, Wierda WG; et al. (2017). "Chronic lymphocytic leukaemia". Nat Rev Dis Primers. 3: 16096. doi:10.1038/nrdp.2016.96. PMC 5336551. PMID 28102226.


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