Pancreatic kaposiform hemangioendothelioma complicated by Kasabach–Merritt phenomenon: A rare entity

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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...

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Pancreatic Kaposiform Hemangioendothelioma Not Responding to Sirolimus

Background  Kaposiform hemangioendothelioma (KHE) is a vascular tumor frequently associated with Kasabach-Merritt phenomenon (KMP), characterized by severe thrombocytopenia and consumptive coagulopathy. Visceral involvement in KHE is rare. In our recent experience, sirolimus has shown to be an effective treatment in cutaneous KHE, becoming indeed the treatment of choice in KMP. We report a case...

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Pancreatic Kaposiform Hemangioendothelioma Presenting with Duodenal Obstruction and Kasabach–Merritt Phenomenon: A Neonate Cured by Whipple Operation

Aim Kaposiform hemangiondothelioma (KHE) is a rare vascular tumor, commonly associated with Kasaback-Merritt phenomenon characterized by thrombocytopenia and consumptive coagulopathy. We report a case of pancreatic KHE presenting with neonatal duodenal obstruction and Kasaback-Merritt phenomenon. Case Report A full term male baby presented with bile stained vomiting on Day 3 of life. Contrast s...

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Infantile hemangioma or kaposiform hemangioendothelioma?

We read with interest the letter sent by Yoon et al (1) about transarterial embolization of a cervicofacial hemangioma associated with Kasabach–Merritt (KM) syndrome in a premature neonate. There are some facets of the letter that warrant further comments, namely the association of the KM phenomenon with vascular tumors and the use of proper consensus-approved terminology adopted by the Interna...

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Kaposiform hemangioendothelioma of paranasal sinus.

Kapossiform hemangioendothelioma (KHE) of the paranasal sins (PNS) is a rare cause of recurrent epistaxis. To date, only two cases of PNS KHE have been reported in the literature, both occurring in the pediatric population. The case presented here appears to be the first case of PNS KHE occurring in an adult. A 46-year-old white female presented with progressively worsening unilateral recurrent...

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ژورنال

عنوان ژورنال: South African Journal of Radiology

سال: 2019

ISSN: 2078-6778,1027-202X

DOI: 10.4102/sajr.v23i1.1760