Hepatosplenic T-Cell Lymphoma in an Immunocompetent Young Female: A Diagnostic Challenge

Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm, most commonly arises from a small subset of γ/δ T-cell receptor–expressing lymphocytes and forms less than 1% of all non-Hodgkin's lymphomas. HSTCL is more common in adolescent and young adults and has a rapidly progressive clinical course and poor outcome due to its refractoriness to conventional chemotherapy regimens. The rarity of this disease, along with lack of nodal involvement and presenting symptoms that mimic different entities including infectious aetiologies, makes this lymphoma a significant diagnostic challenge. To place emphasis on the challenges encountered in establishing the diagnosis, here in, we present a case of a 29-year-old-female who presented with asymptomatic pancytopenia and massive splenomegaly. After an elaborate workup, and extensive investigations, which all turned out to be negative, a combination of morphologic finding of atypical lymphoid cells in the bone marrow, typical immunophenotypic profile on flow cytometry and the pattern of involvement of the bone marrow biopsy, were a key in diagnoses. The report of this case is an effort to emphasize the high index of suspicion for timely detection of such a rare entity.