Fulminant Acute Liver and Heart Failure from Hepatosplenic T-Cell Lymphoma

Abstract

Hepatosplenic T-cell lymphoma is a rare extranodal lymphoma, involving primarily the spleen and liver of young male adults. Diffuse lymphoma cell infiltration can cause moderate liver impairment, yet acute liver or even multiorgan failure is an infrequent initial manifestation.We report on a case of a 71-year-old woman, who presented with acute liver failure, anemia, lymphopenia and thrombocytopenia. Previous medication with clindamycin for diarrhea was suggestive for drug-induced liver failure. However, due to elevated lactate dehydrogenase (LDH), fever, hypoproteinemia, elevated sCD25 and lack of other hepatic disorders including any infectious disease, lymphoma was suspected. On CT scan lymphadenopathy was excluded. Thus, due to hepatosplenomegaly, hepatosplenic T-cell lymphoma was assumed. While histopathological analysis was still pending, the patient´s condition rapidly deteriorated. Cardiorespiratory failure necessitated mechanical ventilation on day 5 after admission. The patient died some hours later in catecholamine-refractory cardiac shock. On autopsy, widespread systemic disease with infiltration of heart, liver, spleen, and bone marrow became apparent with intravascular manifestation in the kidneys and lungs. Infiltration of the myocardium had resulted in acute cardiac failure. As a characteristic of hepatosplenic T-cell lymphoma, molecular pathology and cytogenetic analysis revealed a monoclonal γ T-cell receptor and an immunofluorescence staining compatible with isochromosome 7q in 20% and trisomy of chromosome 8 in 35% of tumor cells.