A Severe Bleeding Diathesis in a 6-Year-Old Girl Secondary to a Composite Diagnosis of Splenic Hemangiomatosis and Small Bowel Lymphangiomatosis

作者：Elder Patrick T^*; Cairns Carole; Dick Alistair; McCallion William A; McCarthy Anthony; Smith Owen P; Macartney Christine

来源：Journal of Pediatric Hematology/Oncology, 2014, 36(5): 404-406.

DOI：10.1097/mph.0000000000000012

• thrombocytopenia
• coagulopathy
• hyperfibrinolysis
• hemangiomatosis
• lymphangiomatosis

A 6-year-old girl presented with presumed relapse of childhood immune thrombocytopenia. Investigations revealed deranged coagulation parameters, abnormal small bowel thickening, and splenomegaly. A clinically significant bleeding diathesis emerged which was refractory to most hemostatic interventions. Laparatomy revealed a composite diagnosis of splenic hemangiomatosis and small bowel lymphangiomatosis. Splenectomy resulted in complete resolution of the coagulopathy. The diagnosis and management of these conditions is inherently complex and without clear guidance. We discuss our perioperative management of the bleeding diathesis. There is a need for long-term follow-up of the underlying pathologies particularly as potentially useful therapeutic agents have emerged.

• 出版日期2014-7