Acute Fulminant Hepatic Failure Due to Hepatosplenic T-cell Lymphoma Mimicking Infiltrative Chronic Myelomonocytic Leukemia

Abstract

Background: Hepatosplenic T-cell lymphoma (HSTL) was reported as a distinct lymphoma entity by Farcet et al with poor prognosis and almost 100% mortality within 2 years. HSTL commonly presents with pancytopenia. This report describes a rare presentation of a case of HSTL with leukocytosis, acute fulminant hepatic failure and bone marrow picture further confusing with chronic myelomonocytic leukemia (CMML). Case Report: A 58-year-old Caucassian male with mild mental retardation presented with bilateral swelling of lower extremities. Initial blood work-up showed leukocytosis with left-shift, thrombocytosis and elevated liver enzymes. The patient's previous documented blood picture obtained 4-5 years earlier was normal. Preliminary bone marrow biopsy was hypercellular and suggestive of either myelodysplastic syndrome or CMML with a possible progression to acute leukemia. Contrary to the initial suspicion of hepatic infiltration of CMML, ultrasound guided liver biopsy showed leukemic infiltration (Figure) into hepatic sinusoids with minimal cholestasis. Specific immunostaining of bone marrow revealed predominance of CD3+ T cells with negativity for other cell surface markers. Genetic analysis of bone marrow demonstrated trisomy 8 and isochromosome 7q, thus strongly suggesting HSTL. After discussing the prognosis and treatment options, patient and family members opted for hospice care and comfort measures. Conclusion: Hepatosplenic T cell lymphoma although predominantly presents with pancytopenia and thrombocytopenia, could also present with leucocytosis and thrombocytosis. Sinusoidal infiltration of liver with hypercellular marrow should raise a high suspicion of hepatosplenic T cell lymphoma.Figure. CD3: staining of showing T-cell predominance.