Kasabach–Merritt Syndrome: Identification of Platelet Trapping in a Tufted Angioma by Immunohistochemistry Technique Using Monoclonal Antibody to CD61

Abstract

Kasabach–Merritt syndrome (KMS) consists of large and rapidly growing vascular tumors associated with thrombocytopenia, generalized petechiae, and bleeding. The cause of the thrombocytopenia is thought to be related to the trapping of platelets by the abnormal endothelium of the tumor. We report an infant with KMS that developed in association with a large tufted angioma. In this case we directly demonstrated platelet trapping in the vascular lumen of the tumor by an immunohistochemical technique using a monoclonal antibody against CD61, a marker of platelets and megakaryocytes.

Kasabach-Merritt Phenomenon: Classic Presentation and Management Options

“…6 Platelet trapping has been directly illustrated by positive immunohistochemistry for CD61, a marker of platelets and megakaryocytes, within the vascular lumen. 10 Similarly, studies also demonstrate localized consumption of fibrinogen when radiolabeled fibrinogen is infused in the setting of KMP. 11 …”

Giant cavernous haemangioma with Kasabach-Merritt syndrome: a case report and review

“…The hypothesis that thrombocytopenia might be caused by localised consumption of platelets in haemangiomas is supported by the immunohistochemical identification of platelet trapping in angiomas using monoclonal antibodies against CD61, a marker of platelets [24], detection of haemangiomatous sequestration of 111 Indium-labelled platelets [21] and in vivo mouse models inducing haemangiomas by transgenic endothelial cells [7]. The predominant histopathological findings of vascular lesions in KMS are a tufted angioma or a kaposiform haemangioendothelioma in association with lymphatic-like vessels and less frequently infantile (juvenile) haemangiomas [1].…”

Treatment of Kasabach–Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon

“…The objectives of treatment of KMS are to prevent bleeding from thrombocytopenia and consumptive coagulopathy and to induce vascular tumor regression. Thrombocytopenia in KMS is due to peripheral destruction, as platelets are trapped in these vascular tumors 14,15 . Platelet transfusions can also exacerbate bleeding 14 .…”