Myeloid sarcoma (MS) is an extramedullary manifestation of acute myeloid leukemia (AML) that may occur de novo, precede, or accompany AML. Hepatic involvement is rare and poses a diagnostic challenge. We report a 65-year-old man presenting with progressive abdominal distension, massive ascites, and constitutional symptoms. Initial laboratory tests revealed bicytopenia, leukocytosis, circulating immature granulocytes, and a cholestatic pattern on liver function tests. Imaging showed homogeneous hepatomegaly with large-volume ascites. Liver biopsy demonstrated diffuse infiltration by immature myeloid cells, with immunohistochemical positivity for myeloperoxidase (MPO) and CD117. Bone marrow (BM) cytogenetic analysis revealed an isochromosome 17q, supporting the diagnosis of hepatic MS, associated with an underlying clonal myeloid neoplasm. The patient received azacitidine and venetoclax, with no response, followed by cytarabine and daunorubicin, with transient clinical improvement; however, progression to AML occurred within a few weeks. This case highlights the diagnostic challenge of hepatic MS presenting as refractory ascites, and supports consideration of early liver biopsy, with sampling from multiple sites, in patients with unexplained ascites and underlying hematologic abnormalities.
Mousadik et al. (Thu,) studied this question.