Multiple spontaneous hematomas with gingival bleeding in the child

Specialty: Pediatrics.

• Purpuric hemorrhagic syndrome
• suspected thrombocytopenia or coagulopathy
• pediatric hemorrhagic diathesis

Why it occurs

• Primary Immune Thrombocytopenia or ITP (peripheral autoimmune destruction of platelets induced by IgG antibodies directed against platelet glycoproteins, typically after a previous viral infection in healthy children)
• Acute Lymphoblastic Leukemia or ALL (malignant neoplasm of lymphoid precursors in the bone marrow that infiltrates the hematopoietic tissue producing insufficiency of the three cell series: plateletopenia, anemia and neutropenia)
• Hemophilia A or B or Von Willebrand disease (congenital coagulopathies due to deficiency of factor VIII, IX or Von Willebrand factor that predispose to prolonged bleeding after minimal trauma, intramuscular hematomas and hemarthrosis)
• Adverse drug reaction (valproic acid, beta-lactam antibiotics or non-steroidal anti-inflammatory drugs that induce immune-mediated thrombocytopenia or platelet dysfunction)
• Henoch-Schönlein purpura (although it typically presents with palpable papular petechiae in the lower extremities of vasculitic origin with normal platelets)
• Hemolytic Uremic Syndrome (thrombocytopenia with microangiopathic hemolytic anemia and acute renal failure after bloody diarrhea due to Shiga toxin-producing Escherichia coli).

Initial workup

Complete blood count with platelet count and immediate peripheral blood smear (essential to confirm isolated thrombocytopenia, rule out the presence of schistocytes suggestive of microangiopathy or leukemic blasts suggestive of leukemia). Complete coagulation times: Prothrombin Time (PT), Activated Partial Thromboplastin Time (APTT, prolonged in hemophilias) and Fibrinogen levels. Bone marrow aspiration or biopsy (mandatory indicated before starting treatment with corticosteroids if there is suspicion of leukemia, or if there is involvement of more than one cell line in the blood count).

Standard management

• Intravenous immunoglobulin or IVIG — indicated of choice in ITP with active mucosal bleeding or platelet count < 10,000/mcL to rapidly block the Fc receptors of splenic macrophages; dose of 0.8 to 1 g/kg as a single intravenous infusion
• Prednisone or Methylprednisolone — systemic corticosteroids indicated in ITP to reduce antibody production; dose of prednisone 4 mg/kg/day orally for 3-4 days, or methylprednisolone 30 mg/kg/day IV for 3 days, always after ruling out leukemia by smear or bone marrow biopsy
• Coagulation factor concentrate — Recombinant factor VIII or IX indicated urgently in confirmed hemophilias when acute bleeding is suspected
• Strictly avoid the intramuscular administration of any drug and the use of acetylsalicylic acid or non-steroidal anti-inflammatory drugs that interfere with platelet function.