Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group

Hirokawa, Makoto; Sawada, Ken; Fujishima, Naohito; Nakao, Shinji; Urabe, Akio; Dan, Kazuo; Fujisawa, Shin; Yonemura, Yuji; Kawano, Fumio; Omine, Mitsuhiro; Ozawa, Keiya

doi:10.3324/haematol.11655

Cited by 88 publications

(73 citation statements)

References 18 publications

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“…We have reported that the discontinuation of maintenance CsA therapy is strongly correlated with relapse in idiopathic PRCA patients, 3 and that thymoma-associated PRCA may also be CsA-dependent. 13 In the present study, there were 3 patients who maintained remission after the discontinuation of CY; however, each relapse-free period after the discontinuation was still only 0, 6 and 11 months. Considering that a relapse can occur even 39 months after the discontinuation of CY, these observation periods may be insufficient to conclude that LGL-leukemia can be cured by CY.…”

Section: Mortality and Overall Survivalcontrasting

confidence: 48%

“…As described elsewhere, 3,13 the questionnaires were sent to 109 hematology departments in Japan, and a total of 185 evaluable patients were collected from 45 institutions. Seventy-three patients were classified as having idiopathic PRCA and 112 patients had secondary PRCA.…”

Section: Data Collection Of the Data And Patients' Characteristicsmentioning

confidence: 99%

“…3,13 Relapse was defined as the reappearance of transfusion requirement. In some analyses, the patients were classified according to the agent used for maintenance therapy such as the CY group or the CsA group regardless of the agents used for successful remission induction.…”

Section: Definition Of Response and Data Analysismentioning

confidence: 99%

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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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Section: Mortality and Overall Survivalcontrasting

confidence: 48%

Section: Data Collection Of the Data And Patients' Characteristicsmentioning

confidence: 99%

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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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“…Despite the heterogeneity of its pathogenesis, most PRCA patients respond to monotherapy with CsA [7,37,38]. The reason for the uniform response to CsA and the target of CsA remain unknown.…”

Section: Foxp3mentioning

confidence: 99%

Cyclosporine restores hematopoietic function by compensating for decreased Tregs in patients with pure red cell aplasia and acquired aplastic anemia

et al. 2016

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Most patients with acquired pure red cell aplasia (PRCA) and some with acquired aplastic anemia (AA) respond well to cyclosporine (CsA), but thereafter often show CsA dependency. The mechanism underlying this dependency remains unknown. We established a reliable method for measuring the regulatory T cell (Treg) count using FoxP3 and Helios expression as markers and determined the balance between Tregs and other helper T cell subsets in 16 PRCA and 29 AA patients. The ratios of interferon-γ-producing CD4 + (Th1) T cells to Tregs in untreated patients and CsAdependent patients were significantly higher (PRCA 5.77 ± 1.47 and 7.38 ± 2.58; AA 6.18 ± 2.35 and 8.94 ± 4.06) than in healthy volunteers (HVs; 3.33 ± 0.90) due to the profound decrease in the percentage of Tregs. In contrast, the ratios were comparable to HVs in convalescent CsA-treated AA patients (4.74 ± 2.10) and AA patients in remission after the cessation of CsA treatment (4.24 ± 1.67). Low-dose CsA (100 ng/ml) inhibited the proliferation of conventional T cells (Tconv) to a similar degree to the inhibition by Tregs in a co-culture with a 1:1 Treg/Tconv ratio. The data suggest that CsA may reverse the hematopoietic suppression in PRCA and AA patients by compensating for the inadequate immune regulatory function that occurs due to a profound decrease in the Treg count.

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“…In this symposium, based on a nationwide survey in Japan, current strategies for the treatment of PRCA were introduced in a presidential lecture. Although this issue is not reviewed in this PIH series, relevant articles can be found elsewhere [21,22] as can focused articles on idiopathic [23], thymoma-associated [24], large granular lymphocyte leukemia-associated [25] and lymphoma-associated PRCA [26]. Oligodeoxynucleotides (ODN) are another deserving wonder that does not appear in this PIH series.…”

Section: Heme and Ironmentioning

confidence: 99%

Guest editorial: the seven wonders of erythropoiesis

Sawada

2011

Int J Hematol

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Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group

Cited by 88 publications

References 18 publications

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

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

Cyclosporine restores hematopoietic function by compensating for decreased Tregs in patients with pure red cell aplasia and acquired aplastic anemia

Guest editorial: the seven wonders of erythropoiesis

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