Intrahepatic Mass-Forming Extramedullary Hematopoiesis in a Patient With Sickle Cell Disease: Case Report and Literature Review

Abstract

Extramedullary hematopoiesis (EMH) is characterized by the expansion of hematopoietic tissue outside the bone marrow niche. It occurs when bone marrow function is insufficient and typically presents with generalized hepatosplenomegaly. We describe a rare case of mass-forming EMH that presented as two intrahepatic lesions, radiologically well-demarcated and nonenhancing on computed tomography. On magnetic resonance imaging, the lesions appeared T1 hypointense and T2 hyperintense with restricted diffusion. The lesions were discovered at the surgical workup for acute cholecystitis in a patient with previous history notable for sickle cell disease. Given the radiological concern for a neoplastic process, an ultrasound-guided biopsy was performed and diagnosed histologically as EMH. A review of the current literature detailing the common clinical, radiological, and pathological presentation of EMH is provided.