Presentation of idiopathic thrombocytopenic purpura (ITP) is dependent on degree of thrombocytopenia with no to mild bleeding symptoms, primarily mucocutaneous bleeding. Severe bleeding of other organ systems is a rare complication. Spontaneous hemarthrosis is rare in patients without hemophilia. We report a child presenting with oral and cutaneous petechial lesions and left knee hemarthrosis without trauma. Laboratory findings showed severe thrombocytopenia consistent with ITP. Serologic tests were consistent with Lyme disease. Hemarthrosis was presumed secondary to Lyme disease monoarticular joint inflammation with bleeding exacerbated by severe thrombocytopenia. Hemarthrosis resolved and platelet counts normalized following immunoglobulin infusion, steroid course, and antibiotics.

BACKGROUND: Coagulopathy and thrombosis are well-described complications of asparaginase therapy; however, treatment practices in pediatric hematology oncology patients vary widely as evidence-based guidelines for clinical management of these complications in this population are lacking. OBJECTIVE: To assess management practices of asparaginase-related coagulopathy by pediatric hematology oncology (PHO) providers. DESIGN/METHOD: Email survey sent to 2,327 PHO providers primarily practicing in the United States. RESULTS: Two hundred and eighty-five (12.2%) attending physicians completed the survey. Only 4.6% (n=13/285) routinely prescribe prophylactic anticoagulation during induction chemotherapy for leukemia. Slightly more than half (n=145/250, 50.9%) of all providers perform baseline coagulation studies. Most providers that were surveyed (n= 185/285, 64.9%) only replete coagulant factors if the patient experiences bleeding or bruising. One hundred and thirty (n = 130/285, 45.6%) physicians replace low fibrinogen, and the median replacement was 100 mg/dL (range: 40-200 mg/dL) with the median target of at least 100 mg/dL (range: 50-200 mg/dL). A minority of physicians (n=39/250, 13.7%) replace low antithrombin at a median cutoff activity level of 60% (range 40-100 %) with a median target of 75% (range: 40-125 %). CONCLUSION: There is a significant variation in PHO provider practices for monitoring and management of asparaginase-associated hemostatic derangements. Evidence-based guidelines have the potential to standardize practices.