An Unusual Cause of Chest Pain: Kasabach-Merritt Syndrome

Abstract

Kasabach-Merritt syndrome (KMS) is a rare complication of hemangiomas that includes both thrombocytopenia and consumptive coagulopathy. Initial cases reported in the pediatric population in the 1940s predominantly involved tufted hemangiomas and kaposiform hemangioendotheliomas. However, this syndrome also includes visceral hemangiomas in adults and has been associated with a broad range of mortality. Abnormal platelet-endothelial interactions lead to platelet sequestration and coagulation factor consumption as a result of these large vascular lesions. Resultant bleeding and thrombosis presents as a localized mimicker of disseminated intravascular coagulopathy (DIC) and can progress to cause compression of nearby organs. Importantly, adults with visceral involvement are often unaware of the presence of these hemangiomas at time of diagnosis and management must be carefully selected with the patient. A multidisciplinary approach including corticosteroids, chemotherapeutics, interferon alpha, antiplatelet agents, embolization, and surgical excision have each been employed in efforts to treat KMS. We present a case of a 36 year-old male with past medical history of hypertension with new-onset chest pain and left arm numbness. He had no nausea, vomiting, melena, or hematochezia. He had no personal or family history of bleeding or clotting disorders. Vital signs were within normal limits and physical examination was unremarkable. After negative serial cardiac enzymes and stable electrocardiograms, Computed tomography (CT) evaluation of the chest was notable for multiple liver lesions. Further evaluation with magnetic resonance imaging (MRI) of the abdomen revealed multiple hepatic hemangiomas with the largest encompassing 15 cm in diameter. Laboratory studies were pertinent for thrombocytopenia, elevated fibrin split products, and a “clotted” PT, PTT, and fibrinogen study. Transaminases, bilirubin, viral hepatitis panel and urine drug screen were negative. The patient's atypical chest pain resolved and was likely attributed to the large liver hemangiomas. Surgery consultation recommended close outpatient follow up to further discuss elective surgical intervention. This case illustrates the importance of maintaining occult etiologies, such as KMS, in the differential of seemingly-routine chief complaints. Medical awareness of this rare syndrome is essential for follow-up, treatment, and prevention of potential life-threatening complications.Figure 1Figure 2Figure 3