BACKGROUND: Kasabach-Merritt syndrome (KMS) is a consumptive coagulopathy associated with the presence of a large vascular lesion. It is often a frustrating condition to treat and it carries a high mortality rate. There are currently no known treatment guidelines. Kasabach-Merritt syndrome is associated with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA); these lesions, when associated with KMS, are locally invasive, aggressive vascular tumors. Treatment options include supportive care, local therapies, and drug and surgical management. In most recent case reports, a multimodal approach to therapy is taken. OBJECTIVE AND CONCLUSION: The objective of this article is to provide a comprehensive review of KMS and give an up-to-date summary of treatment options. The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed.
BACKGROUND:Kasabach-Merritt syndrome (KMS) is a consumptive coagulopathy associated with the presence of a large vascular lesion. It is often a frustrating condition to treat and it carries a high mortality rate. There are currently no known treatment guidelines. Kasabach-Merritt syndrome is associated with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA); these lesions, when associated with KMS, are locally invasive, aggressive vascular tumors. Treatment options include supportive care, local therapies, and drug and surgical management. In most recent case reports, a multimodal approach to therapy is taken. OBJECTIVE AND CONCLUSION: The objective of this article is to provide a comprehensive review of KMS and give an up-to-date summary of treatment options. The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed.
Authors: Andreas F Mavrogenis; Georgios N Panagopoulos; Andrea Angelini; Jan Lesenský; Christos Vottis; Panayiotis D Megaloikonomos; Zinon T Kokkalis; Vasilios Kontogeorgakos; Pietro Ruggieri; Panayiotis J Papagelopoulos Journal: Eur J Orthop Surg Traumatol Date: 2017-06-05