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 fibri...

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Autores principales: Grecia V. Vivas-Colmenares, Gema L. Ramirez-Villar, Jose A. Matute de Cardenas, Jose Bernabeu-Wittel, Israel Fernandez-Pineda
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Publicado: Mattioli1885 2015
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Acceso en línea:https://doaj.org/article/e91ec3558c7a47f5bd89f34fd50fc022
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spelling oai:doaj.org-article:e91ec3558c7a47f5bd89f34fd50fc0222021-11-17T08:31:48ZThe importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon10.5826/dpc.0501a182160-9381https://doaj.org/article/e91ec3558c7a47f5bd89f34fd50fc0222015-01-01T00:00:00Zhttp://dpcj.org/index.php/dpc/article/view/865https://doaj.org/toc/2160-9381 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. Management of vascular tumors complicated by KMP is challenging, and it is common for referral centers to receive patients in critical medical condition after multimodality treatment failure of vascular anomalies. Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP. A full-term male newborn with KHE complicated by KMP is reported. Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids. Grecia V. Vivas-ColmenaresGema L. Ramirez-VillarJose A. Matute de CardenasJose Bernabeu-WittelIsrael Fernandez-PinedaMattioli1885articlekaposiform hemangioendotheliomaKasabach-Merritt phenomenonvincristineticlopidineaspirinDermatologyRL1-803ENDermatology Practical & Conceptual (2015)
institution DOAJ
collection DOAJ
language EN
topic kaposiform hemangioendothelioma
Kasabach-Merritt phenomenon
vincristine
ticlopidine
aspirin
Dermatology
RL1-803
spellingShingle kaposiform hemangioendothelioma
Kasabach-Merritt phenomenon
vincristine
ticlopidine
aspirin
Dermatology
RL1-803
Grecia V. Vivas-Colmenares
Gema L. Ramirez-Villar
Jose A. Matute de Cardenas
Jose Bernabeu-Wittel
Israel Fernandez-Pineda
The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon
description 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. Management of vascular tumors complicated by KMP is challenging, and it is common for referral centers to receive patients in critical medical condition after multimodality treatment failure of vascular anomalies. Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP. A full-term male newborn with KHE complicated by KMP is reported. Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids.
format article
author Grecia V. Vivas-Colmenares
Gema L. Ramirez-Villar
Jose A. Matute de Cardenas
Jose Bernabeu-Wittel
Israel Fernandez-Pineda
author_facet Grecia V. Vivas-Colmenares
Gema L. Ramirez-Villar
Jose A. Matute de Cardenas
Jose Bernabeu-Wittel
Israel Fernandez-Pineda
author_sort Grecia V. Vivas-Colmenares
title The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon
title_short The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon
title_full The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon
title_fullStr The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon
title_full_unstemmed The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon
title_sort importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by kasabach-merritt phenomenon
publisher Mattioli1885
publishDate 2015
url https://doaj.org/article/e91ec3558c7a47f5bd89f34fd50fc022
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