Hepatosplenic T-cell Lymphoma Masquerading as Idiopathic Cytopenia

Hepatosplenic T-cell lymphoma (HSTL) is a highly aggressive form of mature T-cell lymphoma, characterised by abnormal proliferation of cytotoxic T cells in the spleen, liver, and bone marrow. It accounts for <1.0% of all non-Hodgkin lymphomas.

We present a case of HSTL in a 32-year-old male who came with pancytopenia, abdominal distension, constitutional symptoms, and splenomegaly. Initial bone marrow examination was misdiagnosed as Myelodysplastic Syndrome (MDS) or Myelodysplastic Syndrome/Myeloproliferative Neoplasm (MDS/MPN). A repeated bone marrow examination showed CD3-positive neoplastic lymphoid cells in the bone marrow intrasinusoidally and immunophenotyping revealed predominance of gamma-delta (γδ) T-cells.

This case highlights the importance of including HSTL in the differential diagnosis when a patient exhibits splenomegaly and pancytopenia even though background dyspoiesis is prominent. This will enable an early diagnosis of this aggressive cancer.