Hepatosplenic T‐Cell Lymphoma

Abstract

We report an uncommon case of 38-year-old male patient with Hepatosplenic T-Cell lymphoma (HSTCL) which is a rare aggressive form of Peripheral T-Cell lymphoma that is characterized by primary extranodal disease with malignant T-cell proliferation in the liver, spleen, and bone marrow. Our patient presented with progressive painless jaundice, weight loss and massive hepatosplenomegaly. The diagnosis was challenging as he required an extensive investigations that ultimately showed the characteristic clinical, histopathologic, and cytogenetic features of hepatosplenic T-cell lymphoma.Keywords Hepatosplenic Á Lymphoma Á Hepatosplenomegaly Hepatosplenic T-Cell Lymphoma (HSTCL) is an uncommon neoplasm that comprises 5% of peripheral T-cell lymphomas. The majority of cases express the cd T-cell receptor, however, recently, a small number of cases have been reported to express the ab T-cell receptor. Typical clinical features of this lymphoma include a predominance of young male patients, an aggressive clinical course, massive hepatosplenomegaly with sinusoidal infiltration of the liver, spleen, and bone marrow, minimal lymphadenopathy, expression or rearrangement of the cd T-cell receptor, and frequent presence of an isochromosome 7q and trisomy 8. This case highlights the importance of considering diagnosis of HSTCL in patients presenting with jaundice and massive hepatosplenomegaly. CaseA 38-year-old Filipino male presented with a history of lethargy, painless jaundice, dark urine, weight loss (5 kg), and progressive abdominal distension over 3-month-period. He denied history of blood transfusion, significant alcohol consumption and high risk behaviors. He had no history of drug ingestion including acetaminophen and use of herbal remedies. On physical examination; he looked well but deeply jaundiced. His vital signs were stable. There were no lymphadenopathy or peripheral stigmata of chronic liver disease. His abdomen was distended with massive hepatosplenomegaly but without clinically detectable ascites.His initial blood work showed: hemoglobin of 122 g/l, WBCs count of 3.8 9 10 9 /l (73% neutrophils, 16% lymphocytes, 3% monocytes, 1% eosinophils), platelets of 114 9 10 9 /l and his peripheral blood film showed mild pancytopenia with mild atypical reactive lymphocytes. The rest of investigations were as follows: albumin 39 g/l, bilirubin (total 63 lmol/l, direct 48 lmol/l), ALT 355 IU/l, alkaline phosphatase 719 IU/l, creatinine 65 lmol/l, prothrombin time 13.5 s. Serology for common hepatotropic viruses including HAV IgG, HBsAg, anti-HCV and quantitative EBV were negative. Screening tests for human immunodeficiency virus, malaria, schistosomiasis, leishmaniasis, tuberculosis, and common metabolic and autoimmune disorders were also negative. CT scan of the abdomen showed massive hepatosplenomegaly with minimal ascites and scattered small mesenteric lymph nodes.