Bleeding disorder resident survival guide

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Bleeding disorder
Resident Survival Guide
Overview
Causes
FIRE
Diagnosis
Treatment
Do's
Don'ts


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Javaria Anwer M.D.[2]
Synonyms and keywords: Coagulopathy, platelet disorders.

Overview

The bleeding disorder can be due to coagulopathy, platelet dysfunction, or vessel pathology. They can be genetic or acquired. History of the site, duration, associated symptoms, and a thorough physical exam to evaluate the type of bleeding (subcutaneous vs deep tissue) are essential to diagnosis. Screening tests include CBC with peripheral smear, platelet count, PT, aPTT, PFA-100.

Causes

Common causes of bleeding (bleeding disorders) are enlisted below.[1][2][3][4]

 
 
 
 
 
 
 
Causes of bleeding disorders
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Platelet disorders
 
 
Coagulopathy
 
 
 
Vessel/ Supporting tissue defect
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Acquired
 
Genetic
 
 
 
 
 
 
 
❑Aging
Corticosteroid use
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Myeloproliferative disorders

Uremia
❑ Drugs (NSAIDs, asprin, clopidogrel, etc.)
Neoplasia
❑ Monoclonal gammopathies
DIC
Ehrlichiosis
❑ Retroviral infection
❑ Snake venom

Cirrhosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Genetic
 
 
Acquired
 
Prothrombotic
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
❑ Prohemorrhagic liver diseases

Vitamin K deficiency
❑ Drug-induced such as warfarin and heparin
❑ Hemodilution and massive transfusion
Disseminated Intravascular Coagulation (DIC)
Immunoglobulin mediated Factor Deficiency (VIII, V, XIII, X)
Hyperfibrinolysis

Venom induced
 
 
 
 
 
 
 
 
 
Hemorrhagic disorders
 
 
 
Hypercoaguable disease
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Factor VIII deficiency

Factor IX Deficiency
Von Willebrand factor deficiency
❑ Factor XI deficiency
❑ Factor II, V, VII, X deficiency (Common Pathway Proteins)

Factor XIII deficiency and fibrinogen deficiency
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Diagnosis

The algorithm illustrates the approach to the diagnosis of bleeding disorder.[1][5][6]
Abbreviations: HEENT: Head, Eyes, Ears. Nose, and Throat exam; CBC: Complete blood count; APTT Partial thromboplastin time; CMP: Comprehensive metabolic panel; LFTs:Liver function tests
Boxes in red signify that an urgent management is needed.

 
 
 
 
 
 
 
 
 
History

Demographics: Patient age, gender,and race to screen for inherited disorders.
Bleeding history:

❑ Onset of bleed: Differentiate between spontaneous vs post-trauma or post-surgery bleed. Post-trauma may suggest an inherited bleeding disorder.
❑ Duration of bleed: Lifelong vs recent. coagulation factor defect.
❑ Type and site of bleed (skin or muscle): Petechiae, purpura, epistaxis, gingival bleeding, and bruises may suggest a vascular or platelet abnormality. Joint or muscle bleed may suggest coagulation factor abnormality.

Past medical history: For the underlying disease. History of blood or blood components transfusion. Childhood history of epistaxis, bleeding post-circumcision, and umbilical stump bleeding may suggest an inherited bleeding disorder.
Past surgical history: May reveal post-surgical bleed such as after a tooth extraction. History of poor wound healing. ❑ Drug history: For the drugs causing bleeding. Distinguish between drug induced thrombocytopenia and idiopathic thrombocytopenic purpura, or other forms of thrombocytopenia.
Family history: Certain bleeding disorders. Consanguineous marriage history.

Gynaecological history: Menorrhagia or hematuria may suggest vascular or platelet abnormality.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Physical exam

Appearance of the patient
Petechie, bruises, or hemorrhages

Vital signs: Temperature; heart rate (tachycardia with regular pulse may demonstrate hypovolemia); respiratory rate, blood pressure (hypotension); and oxygen saturation may be low due to anemia.
❑ Assess the sites and severity of the bleeding.
❑ Assess if the bleeding is due to systemic disorder, local defect, or hemostatic disorder; inherited or acquired; platelet abnormality, coagulation disorder, or vascular defect. ❑ HEENT
Cardiovascular examination
Respiratory examination
Gastrointestinal system exam includes oral examination, abdominal examination, and digital rectal exam.
Extremities exam

❑ Skin exam: Evaluate for the petechie, bruises, hemorrhages. Location, symmetry, and pattern are important.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Screening Labs

CBC with differential
Platelet count
Prothrombin time (PT)
APTT
Peripheral smear
Thrombin time

Bleeding time/ PFA-100
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Soft tissue hematoma, deep internal hemorrhage, hemarthrosis
 
 
 
 
 
 
 
 
 
 
Superficial cutaneous or mucous membrane bleeding
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
PT normal, aPTT prolonged
 
PT prolonged, aPTT normal
 
 
PT prolonged, aPTT prolonged
 
 
Low platelet count
 
 
 
 
Normal platelet count
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
❑ Check thrombin time
 
 
Idiopathic Thrombocytopenic Purpura (ITP)
❑ Hereditary platelet disorder
Bone marrow failure
 
 
 
 
Check PFA-100
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ E01 }}}
 
{{{ E02 }}}
 
Prolonged
 
Normal
 
{{{ E05 }}}
 
{{{ E06 }}}
 
{{{ E07 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Fibrinogen deficiency
Liver disease
Heparin
 
Factor II, factor V, factor X deficiency
Vitamin K deficiency
Liver disease
 
 
 

Management

The following tables illustrates the treatment to common bleeding disorders.[7][8][9][10]

Disorder Labs Treatment
Hemophilia A and Hemophilia B Factor VIII and factor IX assay
Hemophilia C Factor XI assay Fresh frozen plasma
Von Willebrand disease
Factor XI deficiency (Hemophilia C) Fresh frozen plasma, factor XI is rarely required
Microangiopathic hemolytic anemia Platelet count] (also look for the clinical signs such as neurological deficits among TTP and renal dysfunction among HUS patients.
Thrombocytopenia of pregnancy Platelet count
SLE Platelet count RPR
Liver disease LFTs, APTT, platelet count

Do's

  • A study by Wahlberg et al. demonstrated that the patient's perception of his/her own bleeding may be understated or exaggerated, so labs vital in the assessment of bleeding disorders.[11]
  • The initial clinical impression based on the baseline screening tests should direct specialized laboratory tests to save the time, effort and money.[12]

Don'ts

References

  1. 1.0 1.1 Bashawri LA, Ahmed MA (May 2007). "The approach to a patient with a bleeding disorder: for the primary care physician". J Family Community Med. 14 (2): 53–8. PMC 3410146. PMID 23012146.
  2. George JN (April 2000). "Platelets". Lancet. 355 (9214): 1531–9. doi:10.1016/S0140-6736(00)02175-9. PMID 10801186.
  3. Al-Fawaz IM, Gader AM, Bahakim HM, Al-Mohareb F, Al-Momen AK, Harakati MS (May 1996). "Hereditary bleeding disorders in Riyadh, Saudi Arabia". Ann Saudi Med. 16 (3): 257–61. doi:10.5144/0256-4947.1996.257. PMID 17372424.
  4. Bick, Rodger (2002). Disorders of thrombosis and hemostasis : clinical and laboratory practice. Philadelphia: Lippincott Williams & Wilkins. ISBN 978-0397516902.
  5. Hayward CP (2005). "Diagnosis and management of mild bleeding disorders". Hematology Am Soc Hematol Educ Program: 423–8. doi:10.1182/asheducation-2005.1.423. PMID 16304414.
  6. Blanchette VS, Sparling C, Turner C (April 1991). "Inherited bleeding disorders". Baillieres Clin Haematol. 4 (2): 291–332. doi:10.1016/s0950-3536(05)80162-3. PMID 1912663.
  7. Powell JS (April 2009). "Recombinant factor VIII in the management of hemophilia A: current use and future promise". Ther Clin Risk Manag. 5 (2): 391–402. doi:10.2147/tcrm.s4412. PMC 2697540. PMID 19536318.
  8. Kozek-Langenecker SA, Afshari A, Albaladejo P, Santullano CA, De Robertis E, Filipescu DC, Fries D, Görlinger K, Haas T, Imberger G, Jacob M, Lancé M, Llau J, Mallett S, Meier J, Rahe-Meyer N, Samama CM, Smith A, Solomon C, Van der Linden P, Wikkelsø AJ, Wouters P, Wyffels P (June 2013). "Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology". Eur J Anaesthesiol. 30 (6): 270–382. doi:10.1097/EJA.0b013e32835f4d5b. PMID 23656742.
  9. Gopinath R, Sreekanth Y, Yadav M (September 2014). "Approach to bleeding patient". Indian J Anaesth. 58 (5): 596–602. doi:10.4103/0019-5049.144664. PMC 4260306. PMID 25535422.
  10. Meeks SL, Batsuli G (December 2016). "Hemophilia and inhibitors: current treatment options and potential new therapeutic approaches". Hematology Am Soc Hematol Educ Program. 2016 (1): 657–662. doi:10.1182/asheducation-2016.1.657. PMC 6142469. PMID 27913543.
  11. Wahlberg T, Blombäck M, Hall P, Axelsson G (October 1980). "Application of indicators, predictors and diagnostic indices in coagulation disorders. I. Evaluation of a self-administered questionnaire with binary questions". Methods Inf Med. 19 (4): 194–200. PMID 7432180.
  12. Bick, Rodger (2002). Disorders of thrombosis and hemostasis : clinical and laboratory practice. Philadelphia: Lippincott Williams & Wilkins. ISBN 978-0397516902.