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T-cell large granular lymphocyte leukemia associated with myelodysplastic syndrome: a clinicopathologic study of nine
Yang O Huh1, L Jeffrey Medeiros, Farhad Ravandi
1Department of Hematopathology, Unit 72, University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
Insights
This study examines nine patients with T-cell large granular lymphocyte leukemia (T-LGL) and myelodysplastic syndrome (MDS). Findings suggest a potential etiologic link between T-LGL and MDS, as patients with both conditions showed lower hemoglobin and lymphocyte counts.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- T-cell large granular lymphocyte leukemia (T-LGL) and myelodysplastic syndrome (MDS) are distinct hematologic malignancies.
- Co-occurrence of these conditions is rare, prompting investigation into potential shared etiologies.
Purpose of the Study:
- To describe the clinical and molecular characteristics of patients with coexistent T-LGL and MDS.
- To compare T-LGL/MDS patients with those having T-LGL alone to identify differences and explore potential relationships.
Main Methods:
- Retrospective analysis of nine patients diagnosed with both T-LGL and MDS.
- Immunophenotypic and molecular analyses (T-cell receptor gene rearrangement) were performed.
- Comparison of hematologic parameters (hemoglobin, absolute lymphocyte count) between T-LGL/MDS and T-LGL only groups.
Main Results:
- All nine patients presented with anemia; neutropenia and thrombocytopenia were also common.
- Immunophenotyping revealed CD8+ T-cell populations, and molecular analysis confirmed monoclonal T-cell receptor gene rearrangement.
- Patients with T-LGL/MDS exhibited significantly lower median hemoglobin and absolute lymphocyte counts compared to T-LGL only patients (P < .05).
Conclusions:
- The observed frequency of coexistent T-LGL and MDS suggests a potential etiologic relationship rather than mere coincidence.
- Further research is warranted to elucidate the underlying mechanisms connecting these two hematologic disorders.
Abstract:
We describe 9 patients with T-cell large granular lymphocyte leukemia (T-LGL) who also had a myelodysplastic syndrome (MDS). There were 6 men and 3 women with a median age of 60 years (range, 25-74 years). All patients had anemia at initial examination, 7 had neutropenia, and 5 had thrombocytopenia. The median absolute lymphocyte count was 1,300/microL (1.3 x 10(9)/L; range, 700-3,600/microL [0.7-3.6 x 10(9)/L]). Immunophenotypic analysis showed a CD8+ T-cell population, and molecular analysis showed monoclonal T-cell receptor gene rearrangement in every case. The MDS was classified as refractory cytopenia with multilineage dysplasia (RCMD, n = 5), refractory anemia (n = 2), RCMD with ringed sideroblasts (n = 1), and chronic myelomonocytic leukemia (n = 1). We compared the data for these patients with T-LGL/MDS with a group that had only T-LGL. The median hemoglobin level and absolute lymphocyte count were lower in patients with T-LGL/MDS (P < .05). The frequency of coexistent T-LGL and MDS at our institution suggests an etiologic relationship rather than simple coincidence.
