Introduction and importance:
The simultaneous development of Kasabach-Merritt syndrome (KMS) and focal nodular hyperplasia (FNH) in the same individual is rare. This study aims to report the coexistence of KMS and FNH in a 32-year-old female.
A 32-year-old woman presented to the gastroenterology clinic complaining of heavy menses, fatigue, and hematuria. Physical examination revealed multiple ecchymoses on the skin. Abdominal computed tomography scan revealed a large hepatic lesion with features of atypical hemangioma. Complete blood count showed normochromic, normocytic red blood cells, and thrombocytopenia. Surgery was decided based on a clinical diagnosis of KMS resulting from a giant liver hemangioma. Histopathologic diagnosis of the resected lesion was FNH.
KMS, which refers to a giant hemangioma associated with thrombocytopenia, was initially described by Kasabach and Merritt. It occurs in nearly 1% of hemangiomas and primarily affects infants. KMS is a life-threatening condition that could be fatal if adequate, timely treatment is not administered. FNH is a benign hepatocellular neoplasm with a predilection for females.
KMS and FNH are 2 distinct conditions that can rarely co-exist in the same patient. Corticosteroids, radiotherapy, and surgical resection are the primary modalities of treatment.