Pediatric hematology and oncology
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Pediatr Hematol Oncol · Jul 1998
Review Case ReportsSuccessful multimodal therapy for kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: case report and review of the literature.
We present the management challenge provided by a patient with kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon. A female child presented at 14 months of age with an ecchymotic swelling of her right upper arm and axilla. Subsequently, she developed profound thrombocytopenia and hypofibrinogenemia (Kasabach-Merritt phenomenon). ⋯ To achieve involution of the lesion and preserve function of the arm, the following interventions were involved: embolization, systemic interferon, cyclophosphamide, epsilon aminocaproic acid, and compression therapy. The clinical management of this patient was formidable until we arrived at the proper combination of therapies. Multimodal intervention may be required to manage fastidious hemangioendotheliomas of childhood, achieve clinical improvement, and prevent further morbidity.
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A patient suffering from infantile-onset insulin-dependent diabetes mellitus is reported in whom immune pancytopenia (Evans' syndrome) developed at the age of 2 1/2 years. Hepatosplenomegaly, chronic lymphadenopathy, and elevated levels of immunoglobulins G and M were also present. The course of Evans' syndrome was fatal in this patient. The association of Evans' syndrome with other immune disorders is discussed.