Moderate-dose cyclophosphamide in the treatment of relapsed/refractory T-cell large granular lymphocytic leukemia-associated pure red cell aplasia

Peng, Guangxin; Yang, Wenrui; Zhang, Li; Zhou, Kang; Li, Yang; Li, Yuan; Ye, Lei; Li, Jianping; Fan, Huihui; Song, Lin; Zhao, Xin; Wu, Zhijie; Zhang, Fengkui; Jing, Liping

doi:10.1080/10245332.2015.1101977

Cited by 5 publications

(2 citation statements)

References 32 publications

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“…From selected articles ( n = 20), data were collected into a standardized form including publication year, source, number of total patients and divided by sex, number of subjects with autoimmune disorders, hematological malignancies, or cancers, and Vβ usage by flow cytometry ( Table 2 ) [ 41 , 42 , 46 , 75 , 76 , 88 , 89 , 90 , 91 , 92 , 93 , 94 , 95 , 96 , 97 , 98 , 99 , 100 , 101 , 102 , 103 , 104 , 105 , 106 , 107 ]. A total of 533 T-LGL leukemia patients were evaluable for Vβ usage analysis, and sex was available in 524 subjects: 308 of them were males (58.8%) and 216 females (41.2%) with a male:female ratio of 1.4, as already described in smaller cohorts [ 1 ].…”

Section: Literature Searchmentioning

confidence: 99%

“…Studies were included when: (1) the year of publication was between 2011 and 2021; (2) the studies were conducted on humans and reported clinical data and Vβ usage by flow cytometry; and (3) the studies were on T-LGL leukemia (Figure 2). From selected articles (n = 20), data were collected into a standardized form including publication year, source, number of total patients and divided by sex, number of subjects with autoimmune disorders, hematological malignancies, or cancers, and Vβ usage by flow cytometry (Table 2) [41,42,46,75,76,[88][89][90][91][92][93][94][95][96][97][98][99][100][101][102][103][104][105][106][107]. A total of 533 T-LGL leukemia patients were evaluable for Vβ usage analysis, and sex was available in 524 subjects: 308 of them were males (58.8%) and 216 females (41.2%) with a male:female ratio of 1.4, as already described in smaller cohorts [1].…”

Section: Literature Searchmentioning

confidence: 99%

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The Value of Flow Cytometry Clonality in Large Granular Lymphocyte Leukemia

Giudice

D’Addona

Montuori

et al. 2021

Cancers

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Large granular lymphocyte (LGL) leukemia is a lymphoproliferative disorder of mature T or NK cells frequently associated with autoimmune disorders and other hematological conditions, such as myelodysplastic syndromes. Immunophenotype of LGL cells is similar to that of effector memory CD8+ T cells with T-cell receptor (TCR) clonality defined by molecular and/or flow cytometric analysis. Vβ usage by flow cytometry can identify clonal TCR rearrangements at the protein level, and is fast, sensitive, and almost always available in every Hematology Center. Moreover, Vβ usage can be associated with immunophenotypic characterization of LGL clone in a multiparametric staining, and clonal kinetics can be easily monitored during treatment and follow-up. Finally, Vβ usage by flow cytometry might identify LGL clones silently underlying other hematological conditions, and routine characterization of Vβ skewing might identify recurrent TCR rearrangements that might trigger aberrant immune responses during hematological or autoimmune conditions.

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Section: Literature Searchmentioning

confidence: 99%

Section: Literature Searchmentioning

confidence: 99%

The Value of Flow Cytometry Clonality in Large Granular Lymphocyte Leukemia

Giudice

D’Addona

Montuori

et al. 2021

Cancers

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Successful treatment of refractory/relapsed acquired pure red cell aplasia with sirolimus

Long

et al. 2018

Ann Hematol

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Acquired pure red cell aplasia (aPRCA) is a kind of anemia characterized by severe reticulocytopenia and obvious bone marrow erythroblastic cells decreased. Some patients are refractory or intolerant to the first-line therapy (cyclosporine A with/without steroids). The effects of the second-line therapy are not satisfactory and sometimes not available. In this study, we analyzed the efficacy and side effect of sirolimus on refractory/relapsed aPRCA and investigated the possible mechanism of sirolimus on immune regulation. Twenty-one patients with refractory/relapsed aPRCA were enrolled in this study and were administered with sirolimus. Totally, 76.2% of patients responded to the sirolimus with 42.9% complete response during the experimental period. The median time for reaction was 4 months. Side effects were tolerable including infections; mild oral mucositis; sinus tachycardia, the increase of creatinine, transaminase, triglyceride, or cholesterol; and thrombocytopenia. Most patients stayed in remission or remained stable during the follow-up period. Early drug withdrawal may lead to quick relapse. Compared with healthy control, T levels in patients with aPRCA reduced significantly before sirolimus but recovered after successful treatment. Level of T cells correlated with hemoglobin level after effective sirolimus treatment. Thus, sirolimus was effective and tolerable for refractory/relapsed aPRCA. Effective sirolimus treatment may lead to the upregulation of T cells which may partly explain the underlying mechanism.

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Immunosuppression therapy is effective for both acquired tumor-associated and primary pure red cell aplasia: a match pair case-control study

2020

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Moderate-dose cyclophosphamide in the treatment of relapsed/refractory T-cell large granular lymphocytic leukemia-associated pure red cell aplasia

Abstract: The MD-CTX regimen appears efficacious and safe in the treatment of relapsed/refractory T-LGLL patients.

Cited by 5 publications

References 32 publications

The Value of Flow Cytometry Clonality in Large Granular Lymphocyte Leukemia

The Value of Flow Cytometry Clonality in Large Granular Lymphocyte Leukemia

Successful treatment of refractory/relapsed acquired pure red cell aplasia with sirolimus

Immunosuppression therapy is effective for both acquired tumor-associated and primary pure red cell aplasia: a match pair case-control study

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