A case report of CD4− CD8+ CD56− TCRγδ+ T-large granular lymphocytic leukaemia with severe aplastic anemia and a PNH clone

T-large granular lymphocytic leukemia (T-LGLL) is a rare, indolent lymphoproliferative disorder of lymphocytes. Clonal expansion of T cells, driven by dysregulation of Fas-mediated apoptosis following activation, is a hallmark of this disease. In recent years, research on large granular T-cell leukemia has grown substantially compared with the past; however, most reports have focused on TCRαβ + cases, while studies on other subtypes remain limited, and diagnostic experience with coexisting conditions requires further refinement. This study reports, for the first time, a case of CD4 − CD8 + CD56 − TCRγδ + T-LGLL complicated by severe aplastic anemia (AA) and a paroxysmal nocturnal hemoglobinuria (PNH) clone. The case is characterized by positive TCRγδ gene rearrangement, with T-cell–mediated hematopoietic suppression leading to the development of AA and the PNH clone. We hope this study will address a gap in the current literature. Here, we summarize the common pathophysiological mechanisms and guiding treatment principles for these three diseases.

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Publication Details

Journal
Annals of Hematology
Published
2026-06-29
DOI
https://doi.org/10.1007/s00277-026-07141-7
Primary Topic
Chronic Lymphocytic Leukemia Research
Type
article
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article

A case report of CD4− CD8+ CD56− TCRγδ+ T-large granular lymphocytic leukaemia with severe aplastic anemia and a PNH clone

Yufei Du, S.‐Z. Jin, Jinling Zhang
Annals of Hematology
Chronic Lymphocytic Leukemia Research
article

A case report of CD4− CD8+ CD56− TCRγδ+ T-large granular lymphocytic leukaemia with severe aplastic anemia and a PNH clone

Yufei Du, S.‐Z. Jin, Jinling Zhang
article en

Abstract

T-large granular lymphocytic leukemia (T-LGLL) is a rare, indolent lymphoproliferative disorder of lymphocytes. Clonal expansion of T cells, driven by dysregulation of Fas-mediated apoptosis following activation, is a hallmark of this disease. In recent years, research on large granular T-cell leukemia has grown substantially compared with the past; however, most reports have focused on TCRαβ + cases, while studies on other subtypes remain limited, and diagnostic experience with coexisting conditions requires further refinement. This study reports, for the first time, a case of CD4 − CD8 + CD56 − TCRγδ + T-LGLL complicated by severe aplastic anemia (AA) and a paroxysmal nocturnal hemoglobinuria (PNH) clone. The case is characterized by positive TCRγδ gene rearrangement, with T-cell–mediated hematopoietic suppression leading to the development of AA and the PNH clone. We hope this study will address a gap in the current literature. Here, we summarize the common pathophysiological mechanisms and guiding treatment principles for these three diseases.

Annals of Hematology
Yanbian University Hospital (CN)
Good health and well-being
Openalex Percentile: Top 8%
Chronic Lymphocytic Leukemia Research
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