Hepatosplenic T Cell Lymphoma Mimicking Hemophagocytic Lymphomhistiocytosis: A Case Report

Hepatosplenic T cell lymphoma (HSTCL) is rare and aggressive extranodal lymphoma characteristically involves young males in third or fourth decade of life. It frequently manifests as pancytopenia with severe thrombocytopenia, hepatosplenomegaly, and much rarely, hemophagocytic lymphohistiocytosis (HLH).

Medical records of a patient who presented with HLH as a presenting signs of HSTCL were reviewed. Literature search was performed to identify characteristic demographics and natural course of HSTCL reported to date.

A 30-year-old male presented after 6 months of constitutional symptoms. Remarkable findings were pancytopenia, mildly elevated LFT, and high ferritin level (89,000). Extensive work up for autoimmune and infectious etiology was negative. Worsening anemia and thrombocytopenia prompted the third bone marrow biopsy, which revealed the first evidence of hemophagocytosis. Despite of Cyclosporine A and Etoposide, pancytopenia worsened which prompted splenectomy and core needle liver biopsy. Sinusoids of spleen and liver were densely infiltrated with atypical lymphocytes consistent with T cell phenotype. Diagnosis of HSTCL was confirmed after PCR detection of gamma delta T cell receptor rearrangement. Previous bone marrow biopsies were retrospectively reviewed, which revealed small clusters of cells staining positive for CD3. The patient underwent three courses of chemotherapy that included high-dose Cytarabine, Etoposide and adriamycin. Post-chemotherapy course was complicated with disseminated Candidiasis complicated with mycortic aneurysm and worsening pancytopenia. The patient expired due to overwhelming septic shock 6 months after the pathologic diagnoses of HSTCL.

HSTCL causes aberrant expansion of gamma delta T cells and defective innate immunity, and is an important secondary etiology for HLH. Splenectomy has diagnostic significance but little therapeutic benefit. Longer survival was reported in patients who underwent cytarabine-based chemotherapy; however median survivals in anecdotal case series all fall within 2 years regardless of regimen.

No relevant conflicts of interest to declare.