3.3 Case 3: Kaposiform Lymphangiomatosis
A 7-year-old girl with “chronic ITP”, diagnosed at an outside institution, presented with abdominal pain and epistaxis and was found to have splenomegaly, moderate thrombocytopenia, and small amount of ascites on ultrasound. She developed worsening ascites and a rapid decline in hemoglobin and platelets as well as PT and PTT prolongation. Paracentesis was performed revealing grossly bloody peritoneal fluid. Her hematologic abnormalities were initially thought to be due to ITP in combination with acute consumption of platelets and coagulation factors. She was subsequently given packed red blood cell (RBC), fresh frozen plasma and multiple platelet transfusions. Her platelet count and fibrinogen level were noted to initially worsen and then gradually improve. The patient recovered from this event but developed several more episodes of painful ascites, which were likely hemorrhagic, although repeat paracentesis was not performed. Given her presumed diagnosis of ITP, she underwent splenectomy. Pathology revealed diffuse infiltration of her spleen with KLA (Figure 3A-B) .