TY - JOUR
T1 - Successful multimodal therapy for kaposiform hemagioendothelioma complicated by Kasabach-Merritt
AU - Blei, Francine
AU - Karp, Nolan
AU - Rofsky, Neil
AU - Rosen, Robert
AU - Greco, M. Alba
PY - 1998
Y1 - 1998
N2 - 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.
AB - 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.
KW - Hemangioendothelioma
KW - Kasabach-Merritt phenomenon
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U2 - 10.3109/08880019809014013
DO - 10.3109/08880019809014013
M3 - Review article
C2 - 9658430
AN - SCOPUS:0031836890
SN - 0888-0018
VL - 15
SP - 295
EP - 305
JO - Pediatric Hematology and Oncology
JF - Pediatric Hematology and Oncology
IS - 4
ER -