Tufted angioma associated with Kasabach-Merritt phenomenon: a therapeutic challenge.
نویسندگان
چکیده
Fig. 1. (A) Tufted angioma involving the chin in a one-month-old child. (B) The patient at 2 months of age showing a subtle increase in tumour size and decrease in platelet count. (C) A subtle residual lesion on the chin of the patient at the age of 5 years. Tufted angioma (TA) is an infrequently seen, benign vascular tumour, which was first described in 1949 (1). It is characterized clinically by a solitary tumour or infiltrated plaque thought to have a more inflammatory appearance than vascular. The great majority appear during the first 5 years of life; however, rare cases of TA presenting at birth have been noted (2, 3). Kasabach-Merritt phenomenon (KMP) is a form of microangiopathic haemolysis, with coagulopathy related to the sequestration of platelets and coagulation factors within the tumour. The sequestration creates a form of disseminated intravascular coagulation, with the patients having both a propensity to clotting and a high risk of bleeding. It is an infrequent complication of TA, which represents a therapeutic challenge (4).
منابع مشابه
Kasabach-Merritt Phenomenon: Classic Presentation and Management Options
Kasabach-Merritt phenomenon (KMP) is a rare consumptive coagulopathy associated with specific vascular tumors, kaposiform hemangioendothelioma, and tufted angioma. Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia, hypofibrinogenemia, elevated fibrin split products, and rapid tumor growth, can be life-threatening. Severe symptomatic anemia may also be present. With prompt ...
متن کامل[Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon].
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 app...
متن کاملA new successful combination therapy with atenolol and prednisolone for Kasabach-Merritt syndrome
Kasabach-merritt syndrome is a rare life-threatening syndrome associated with vascular tumors such as tufted angioma and kaposiform hemangioendothelioma. For this syndrome, there exist a few treatment modalities with variable results. For many years, corticosteroids have been employed in the first-line therapy. Nowadays, on the other hand, β blockers such as propranolol have been used owing to ...
متن کاملEfficiency of an mTOR Inhibitor in Kasabach-Merritt Phenomenon with Indolent Tufted Angioma: A Case Report.
متن کامل
The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that KMP is not caused by infantile hemangiomas. Man...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Acta dermato-venereologica
دوره 90 5 شماره
صفحات -
تاریخ انتشار 2010