Successful multimodal therapy for kaposiform hemagioendothelioma complicated by Kasabach-Merritt

Francine Blei, Nolan Karp, Neil Rofsky, Robert Rosen, M. Alba Greco

Research output: Contribution to journalReview articlepeer-review

65 Scopus citations


We present the management challenge provided by a patient with kaposiform hemagioendothelioma 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). Biopsy of the lesion revealed kaposiform hemangioendothelioma, which has been reported as the predominant pathologic diagnosis associated with 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 managed fastidious hemangioendotheliomas of childhood, achieve clinical improvement, and prevent further morbidity.

Original languageEnglish (US)
Pages (from-to)295-305
Number of pages11
JournalPediatric Hematology and Oncology
Issue number4
StatePublished - 1998


  • Hemangioendothelioma
  • Kasabach-Merritt phenomenon

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology


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