Hepatic hemangiomas are generally asymptomatic and rarely cause symptoms of consumptive coagulopathy and thrombocytopenia. We present a case of Kasabach-Merritt Syndrome in a patient found to have extensive hepatic hemangiomatosis. A 37-year-old male with a medical history of an abdominal hernia repair and an essential tremor presented with worsening fatigue, increased bruising, and abdominal distention over a 4-month period. Labs were significant for a hemoglobin of 8.4 g/dL, total bilirubin of 1.5 mg/dL, platelet count of 70,000/uL, INR 2.3, and fibrinogen level of less than 60 mg/dL. Abdominal magnetic resonance imaging (MRI) revealed variably sized lesions representing hepatic hemangiomas that replaced a majority of his hepatic parenchyma (Figure 1). Based on abnormal imaging and laboratory data revealing consumptive coagulopathy and hemolysis, a diagnosis of Kasabach-Merritt syndrome (KMS) was made. He was started on high dose methylprednisolone and transfused aggressively with packed red blood cells, cryoprecipitate, prothrombin complex concentrate, and fibrinogen concentrate with minimal improvement in his consumptive coagulopathy. Given his extensive hepatic dysfunction, he was listed for liver transplantation. He remained clinically stable with no signs of active bleeding and was discharged home. Two months after initial diagnosis, he underwent a successful liver transplant. His liver explant weighed 6.7 kg and measured 28cm in the right lobe and 30 cm in the left lobe. Kasabach-Merritt Syndrome is a rare but serious complication defined by the triad of hemangioma, consumptive coagulopathy, and thrombocytopenia. The incidence of this syndrome in adults is unknown and the majority of cases reported in children. Due to its rarity, there are no clinical guidelines for the treatment of this syndrome and the general approach is the correction of the hematologic derangements. Other cases reported the successful use of corticosteroids, antiplatelet therapy, and chemotherapeutic agents. Primary treatments of hepatic hemangiomas consist of embolization, enucleation, and resection with surgery reserved for symptomatic or complicated cases. Among previous case reports, the majority of giant liver hemangiomas (diameter greater than 20cm) underwent transplant, with one undergoing enucleation, and proved curative. Given his extensive hemangiomatosis, liver size, and coagulopathy refractory to medical therapy, a liver transplant was performed and proved curative.2414 Figure 1. Magnetic resonance imaging revealing multiple hepatic lesions without evidence of cirrhosis.
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