Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon - 21/08/11
Boston, Massachusetts; New York, New York; and San Francisco, California
Abstract |
Kasabach-Merritt phenomenon is a serious coagulopathy associated with kaposiform hemangioendothelioma (KHE), tufted angioma, and possibly other vascular neoplasms. KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia. We retrospectively reviewed 10 cases of KHE without Kasabach-Merritt phenomenon. The tumors appeared as soft tissue masses with the overlying skin being either normal, erythematous, or violaceous. There were no radiologic or microscopic differences in noncoagulopathic KHE as compared with coagulopathic KHE. Evidence of platelet trapping and hemosiderin deposition was seen histologically, despite normal serum platelet levels. All KHE were less than 8 cm in diameter, suggesting that tumors that grow no larger than this size are less likely to trap platelets in sufficient quantity to cause thrombocytopenia. Our series confirms that KHE appears with a wide spectrum of behavior and response to treatment. The decision as to whether or not to treat a noncoagulopathic KHE should be based on the size and location of the tumor and the possible side effects of therapy.
Le texte complet de cet article est disponible en PDF.Abbreviations used : CS, KHE, KMP, TA, VCR
Plan
Funding sources: None. Conflicts of interest: None identified. Reprints not available from the authors. |
Vol 52 - N° 4
P. 616-622 - avril 2005 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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