Two Cases Of Large Cutaneous Vascular Tumors With Kasabach-Merritt Phenomenon And Review Of Literature

IntroductionKasabach-Merritt phenomenon (KMP) is a rare complication of pre-existing he mangioma in infancy and early childhood Patients with KMP present with an e nlarged hemangioma and laboratory features of thrombocytopenia and consumpti ve coagulopathy. Although a recent study showed spontaneous regression in mo st cases within 2 years, these benign tumors may develop potentially fatal KM P as complication. With an overall mortality rate of 20-30%, various treatment regimens have been introduced to manage KMP. Recent reports recommended a careful stepwise approach in the management of KMP, but the results demo nstrated inconsistent efficacy. Among the regimens recommended systemic corti costeroid is considered as first-line therapy for KMP patients because of its lo w cost and ease of administration.ObjectiveTo understand the clinical manifestation, the diagnosis, and treatment of Kasab ach—Merritt phenomenon(KMP).Methods(1)We treated a patient who was diagnosed as KMP and received chemotherap y including Systemic Corticosteroid and antibiotic in our hospital in July of 20 10.(2)Another patient who was diagnosed as KMP and received surgical resection following chemotherapy which is not responded to the clinical characteristics,1 aboratory test, therapy and response to chemotherapy and surgery were analyze d.The related literature was also reviewed.ResultsAfter chemotherapy protocol included Systemic Corticosteroid and antibiotic the platelets returned to normal level after 10-days’treatment.The other patient w as unresponsive to usual medical treatments, so surgical resection was consider ed. Following surgery, coagulation parameters normalized within two weeks, al ong with the decrease in size of the tumor,and the infant improved after 3-we eks’ treatment.ConclusionKaposiform hemangioendothelioma and tufted angioma in infancy may present with KMP and require aggressive treatment. Management of KMP has been ch allenging because of its rarity and there are no well-established systematic treat ment strategies to date. Systemic corticosteroid is considered to be first-line the rapy in patients with KMP. If the patient is not responded to medical manage ment, surgical resection should be considered...