Kasabach-Merritt Syndrome-Associated Kaposiform Hemangioendothelioma Successfully Treated With Cyclophosphamide, Vincristine, and Actinomycin D

Abstract

A discussion of congenital and familial erythrocytosis is presented, and a review of the literature regarding the possible mechanisms causing erythrocytosis is included.

Clinical Review: Management of Adult Kasabach-Merritt Syndrome Associated with Hemangiomas

“…The general approach is to manage coagulopathy effectively with supportive care and medical management [23][24][25][26][27][28][29][30] …”

“…mTOR is regulated by phosphoinositide-3-kinase (PI3K) [67]. The mTOR inhibitor sirolimus has been shown to inhibit lymphangiogenesis, and it has been successfully used as a treatment option for vascular anomalies in children [28,[68][69][70]. Advantages of sirolimus include oral administration without the need for a central line and more rapid…”

Benign Cutaneous Vascular Tumors of Infancy

“…99 Isolated cases of improvement with vincristine therapy, either alone or in combination with other chemotherapeutic agents, have also been reported. 55,100,101 Therapeutic guidelines for the treatment of TAs have also yet to be established. Like KHE, smaller lesions may be amenable to surgery and treatment of larger lesions with systemic corticosteroids and/or interferon alfa has shown mixed results.…”

Massive Congenital Vascular Tumor of Lower Limb: Kaposiform Hemangioendothelioma

“…[7] The treatment of associated KMS is more troublesome and may need additional multimodal therapy approach in form of steroids, interferon and cytotoxic drugs. [8] Steroids and interferon are usually favored, though no strict guidelines available owing to its rarity. Low dose radiotherapy, angio-embolisation, chemo-embolisation, and intralesional bleomycin/ ethanol has also been described recently in literature.…”