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