Background We are describing an interesting case that challenged our interpretation between large granular lymphocytic leukemia (LGL) and gamma delta T-cell lymphoma. LGL is a rare leukemia with a median age onset at 55 years having characteristic morphology, multiple autoimmune disorders, and an indolent clinical course in females, whereas gamma delta positive T-cell lymphoma is an uncommon aggressive non-Hodgkin's lymphoma variant found in young males.
Case Presentation A 53-year-old female with a 3-year history of pancytopenia, recurrent upper respiratory infections, and lupus presented with worsening cytopenia. CT scan of the chest, abdomen, and pelvis showed diffuse hepatosplenomegaly with minimal lymphadenopathy. Peripheral blood smear showed pancytopenia with a minor population of large granular lymphocytes (WBC 1.1 L, RBC 3.2 L, Hb 11 L, platelets 86 L, neurophil 0.1 L, lymph 80 H). Bone marrow biopsy revealed 40 to 50% cellularity with involvement by T-cell lymphoproliferative disorder of TCR-gamma/delta immunophenotype; positive for CD2, CD3, CD5 dim, and CD7dim and negative for CD4, CD8, CD16, CD25, and CD56, with variable CD57 expression. Molecular studies for T-cell receptor gene rearrangement showed no evidence of T-cell clonal population. The cytogenetics were normal diploid. Since the patient had features of both LGL and T-cell gamma delta lymphoma, the case was referred to M.D. Anderson Cancer Center for a second opinion, where LGL was the final diagnosis. Patient was treated with cyclosporine and she responded well to it, resolving her pancytopenia.
Discussion In view of the characteristics of these rare disorders our patient was CD8 negative and CD3 positve. CD8 negativity is not consistent with LGL, yet she had systemic signs and symptoms, including fatigue, sweats, recurrent infections, and arthritis, which are main clinical features of T-cell LGL. In addition, CT scan findings of mild hepatosplenomegaly, which is observed in 20%, and IgM and IgG monoclonal gammopathy, which is present in 8% of patients, supported the diagnosis of LGL. A female patient with an indolent clinical course and 72% lymphocytes in the peripheral blood also argues against the features of T-cell gamma delta lymphoma. This case highlights the clinical spectrum of LGL proliferations and T-cell gamma delta lymphoma and how immunophenotypic and genotypic studies can help establish the diagnosis and modify the treatment.
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