Smoldering γδT-Cell Granular Lymphocytic Leukemia Associated with Pure Red Cell Aplasia

Mori, Shunsuke; Suzushima, Hitoshi; Nishikawa, Kouji; Miyake, Hirosada; Yonemura, Yuji; Tsuji, Norihide; Kawaguchi, Tatsuya; Asou, Norio; Kawakita, Makoto; Takatsuki, Kiyoshi

doi:10.1159/000203967

Cited by 12 publications

(4 citation statements)

References 5 publications

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“…An extensive review of previous reports shows that a total of 24 cases with γ δ T‐LDGL, including our report, have been identified (4, 5, 14, 22–24, 34–45). Fourteen of them were Japanese, although no cases were reported from other parts of Asia.…”

Section: Discussionmentioning

confidence: 64%

“…The clinical findings and hematological data, such as large granular lymphocytosis, cytopenia, autoimmunological abnormalities, and a clinical course of several years, seem to be specific characteristics of γ δ T‐LDGL. The elevated levels of NK cell activity and ADCC or the suppressive effect of peripheral blood on colony formation were identified in several γ δ T‐LDGL cases (34–36, 41). In patient 1 with neutropenia, a semisolid colony assay showed that coculture of PBMC with Bone Marrow Mononuclear Cells (BMMNC) suppressed Colony Forming Unit‐granulocyte Macrophage (CFU‐GM) formations (data not shown).…”

Section: Discussionmentioning

confidence: 99%

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Lymphoproliferative disease of granular lymphocytes with T‐cell receptor gamma delta‐positive phenotype: restricted usage of T‐cell receptor gamma and delta subunit genes

Makishima

Ishida

Saitô

et al. 2003

European J of Haematology

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Lymphoproliferative disease of granular lymphocytes (LDGL) is characterized by more than 0.5 x 109/L of proliferating granular lymphocytes in the peripheral blood. Because of its rarity, the characteristics of LDGL with T-cell receptor (TCR) gammadelta phenotype (gammadeltaT-LDGL) have not yet been identified. This report describes the clinical, hematological, and immunological findings of four patients with this disease. In two cases, the clinical course was indolent and the other two patients required various therapies. The cells had a common immunophenotype: CD3+, CD4-, CD16+, CD56-, CD57-, CD122-, TCR-gammadelta+, and three were CD8-positive. The immunopurified TCR-gammadelta cells from the patients expressed only Vgamma9 and Vdelta1. Spectratyping and sequencing showed mono- or oligoclonality for TCRgamma and TCRdelta subunit genes. Soluble Fas ligand in sera was significantly elevated in all patients. These findings suggest that gammadeltaT-LDGL qualifies as a distinct disease entity.

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 99%

Lymphoproliferative disease of granular lymphocytes with T‐cell receptor gamma delta‐positive phenotype: restricted usage of T‐cell receptor gamma and delta subunit genes

Makishima

Ishida

Saitô

et al. 2003

European J of Haematology

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“…Case 13 has been previously reported. 15 A unique patient number was given at each participating institution to protect individual information. This study was approved by the institutional review board, and performed according to the declaration of Helsinki and the ethical guidelines for epidemiological research of the Ministry of Education, Culture, Sports, Science and Technology, and the Ministry of Health, Labour and Welfare of Japan.…”

Section: Data Collection Of the Data And Patients' Characteristicsmentioning

confidence: 99%

Long-term responses and outcomes following immunosuppressive therapy in large granular lymphocyte leukemia-associated pure red cell aplasia: a Nationwide Cohort Study in Japan for the PRCA Collaborative Study Group

Fujishima¹,

Sawada²,

Hirokawa³

et al. 2008

Haematologica

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Large granular lymphocyte leukemia-associated pure red cell aplasia accounts for a significant portion of secondary pure red cell aplasia cases. However, because of its rarity, long-term responses and relapse rates after immunosuppressive therapy are largely unknown. We conducted a nationwide survey in Japan and collected 185 evaluable patients. Fourteen patients with large granular lymphocyte leukemia-associated pure red cell aplasia were evaluated. Cyclophosphamide, cyclosporine A and prednisolone produced remissions in 6/8, 1/4 and 0/2 patients respectively. Seven and 5 patients were maintained on cyclophosphamide or cyclosporine A respectively. Two patients relapsed after stopping cyclophosphamide, and 2 patients relapsed during maintenance therapy with cyclosporine A. The median relapse-free survival in the cyclophosphamide -and the cyclosporine A groups was 53 and 123 months respectively. Large granular lymphocyte leukemia-associated pure red cell aplasia showed a good response to either cyclophosphamide or cyclosporine A. Most patients continued to receive maintenance therapy and it remains uncertain whether cyclophosphamide or cyclosporine A can induce a maintenance-free hematologic response in large granular lymphocyte leukemia-associated pure red cell aplasia.

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“…These LGL expansions have been defined as neoplastic processes because of their clonal nature. Most T-LGLL is of the αβ type, but some cases of T-LGLL were of the γδ type 72,73. γδT-LGLL is rare, and its association with PRCA is also rare.…”

Section: Types Of Lgl Lymphocytosismentioning

confidence: 99%

<p>Pathophysiologic Mechanisms And Management Of Large Granular Lymphocytic Leukemia Associated Pure Red Cell Aplasia</p>

Qiu¹,

Qin²,

Tian³

et al. 2019

OTT

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Large granular lymphocytic leukemia (LGLL) is a chronic clonal lymphoproliferative disease of mature T or NK cells, and produces a variety of hematological abnormalities. Pure red cell aplasia (PRCA) is a rare haematological disease and is one of the most common complications of LGLL. LGLL-associated PRCA may represent a relatively indolent type and may be more common than reported, but its natural history and clinical course have not been well described. The ethnic origin of the patients is an important consideration in determining the relationship between PRCA and LGLL. Guidelines and progresses for management of LGLL-associated PRCA rely on accumulation of empirical experiences, integrative analyses of several cases and clinical trials. The purpose of this review is to evaluate occurrence, possible mechanisms, diagnosis, clinical features, treatments and outcomes of LGLL-associated PRCA.

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Smoldering γδT-Cell Granular Lymphocytic Leukemia Associated with Pure Red Cell Aplasia

Cited by 12 publications

References 5 publications

Lymphoproliferative disease of granular lymphocytes with T‐cell receptor gamma delta‐positive phenotype: restricted usage of T‐cell receptor gamma and delta subunit genes

Lymphoproliferative disease of granular lymphocytes with T‐cell receptor gamma delta‐positive phenotype: restricted usage of T‐cell receptor gamma and delta subunit genes

Long-term responses and outcomes following immunosuppressive therapy in large granular lymphocyte leukemia-associated pure red cell aplasia: a Nationwide Cohort Study in Japan for the PRCA Collaborative Study Group

<p>Pathophysiologic Mechanisms And Management Of Large Granular Lymphocytic Leukemia Associated Pure Red Cell Aplasia</p>

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