Approach to a patient with bleeding disorder

Bleeding/bruising in children:

  • Accidental
  • Non-accidental injury/Abuse
  • Bleeding diathesis

 

Common Causes of bleeding in a well child

  • ITP
  • Henoch-Schonlein purpura
  • Hemophilia A or B
  • von Willebrand disease
  • Vitamin K deficiency
  • Warfarin or heparin

Causes of bleeding in a Sick child

  • DIC
  • Severe liver dysfunction
  • Acute leukemia
  • Neuroblastoma
  • Lymphoma
  • Meningococcal infection

History

  • Site of bleeding
    • Localized: e.g. epistaxis from single nostril (FB/trauma)
    • Generalized: a bleeding disorder
  • Onset of bleeding
    • Days to weeks (acute) – acquired (ITP, HSP, meningococcemia)
    • Over a longer period of time – hereditary cause (hemophilia A, B, and von Willebrand disease)
  • Site of bleeding
    • Mucosal (epistaxis, gums, and menorrhagia) and petechiae and bruising: platelet disorders and vWD
    • Deep muscle and joints: hemophilia
  • Drug intake
    • NSAIDs: inhibit platelet function
    • Anticonvulsants: thrombocytopenia
  • Past History
    • Bleeding from the umbilical stump, after immunizations, trivial trauma, dental extractions, and surgery – (hemophilia A, B, and vWD)
  • Family History
    • Primarily males affected – hemophilia
    • Menorrhagia in female relatives – vWD

Physical Examination

  • Petechiae vs mosquito bites (blanch on pressure)
  • Symmetrical distribution of petechiae over extensor surfaces: HSP
  • Periorbital ecchymoses (raccoon eyes) - neuroblastoma
  • Ecchymoses over flanks, abdomen, and back: a bleeding disorder
    Consider abuse in unusual patterns of bruising or bleeding
  • Swollen tender joint - hemophilia, HSP, acute leukemia, neuroblastoma
  • Pallor – leukemia, DIC and blood loss
  • Icterus - the liver disease with failure
  • Lymphadenopathy – leukemia, lymphoma
  • Hepatosplenomegaly – leukemia, lymphoma

Laboratory Investigations

  • Complete blood count (CBC)
    • Platelet count
      • Thrombocytopenia: ITP, DIC
      • Normal: HSP
    • Leucopenia with anemia and thrombocytopenia: leukemia, aplastic anemia, and lymphoma
  • Peripheral smear exam
    • For platelet count and morphology
    • Blast cells: leukemia
  • Prothrombin time (PT) – evaluates extrinsic (factor VII) and common pathway (factors X, V, and II)
  • Activated partial thromboplastin time (aPTT) – evaluates intrinsic (factors VIII, IX, XI, XII and vWF) and common pathway
  • Thrombin time: prolonged in hypo/dysfibrinogenemia and heparin contamination
  • Fibrinogen level decreased – hypo/dysfibrinogenemia, DIC, and severe liver disease
  • Factor VIII level < 40% - Hemophilia A
  • Factor IX level < 40% - Hemophilia B

Clinical Scenarios in Bleeding Disorders

  • Significant bleeding history, normal PT, normal aPTT, and normal platelet count
    • vWD, HSP, platelet function disorders
  • History of bleeding and isolated prolongation of the aPTT
    • Hemophilia A and B
  • History of bleeding and isolated prolongation of the PT
    • acute liver failure and warfarin ingestion
  • History of bleeding and prolongation of both PT and aPTT with thrombocytopenia
    • DIC and liver failure
    • A child with petechiae and/or mucosal bleeding associated with isolated thrombocytopenia
    • ITP

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