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) .