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.
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.
Authors
Raghvan Raghvan, Wee Wee, Liam Liam, Mohd Shakri Mohd Shakri, Ho Ho, Jamian Jamian, Subbiah Subbiah, Khalid Khalid
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