Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A. A nationwide cohort study in Japan for the PRCA Collaborative Study Group

Sawada, Kenichi; Hirokawa, Makoto; Fujishima, Naohito; Teramura, Masanao; Bessho, Masami; Dan, Kazuo; Tsurumi, Hisashi; Nakao, Shinji; Urabe, Akio; Omine, Mitsuhiro; Ozawa, Keiya

doi:10.3324/haematol.11192

Cited by 83 publications

(86 citation statements)

References 21 publications

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“…Sawada et al reported that discontinuation of maintenance cyclosporine therapy was strongly correlated with relapse in patients with idiopathic PRCA. 11 As for idiopathic PRCA, remissions from thymoma-associated PRCA may also be cyclosporine-dependent. If this is true, other therapeutic modalities may be required to cure thymoma-associated PRCA.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2008

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BackgroundThymoma-associated pure red cell aplasia (PRCA) accounts for a significant proportion of cases of secondary PRCA and immunosuppressive therapy has been reported to be useful in this condition. However, because of its rarity, the long-term response and relapse rates after immunosuppressive therapy are largely unknown, and optimal management of this disorder remains unclear. The aim of this study was to collect more information on the outcome of patients with thymoma-associated PRCA.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2008

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“…Despite the various immune mechanisms that underlie erythroid progenitor cell suppression, most PRCA patients respond to cyclosporine (CsA), an immunosuppressant which selectively suppresses T cells. Even more characteristically, PRCA patients show a strong dependency on CsA: they usually require low dose CsA to maintain good erythropoietic function [7]. This is in sharp contrast to the response to CsA in patients with idiopathic aplastic anemia (AA), most of whom can be withdrawn from CsA after achieving remission with antithymocyte globulin (ATG) and CsA therapy [8].…”

Section: Introductionmentioning

confidence: 70%

“…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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“…The acquired form of PRCA has been reported to be caused by parvovirus infection, collagen disease, leukemia, lymphoma, thymoma, or treatment with ESAs or other drugs (such as phenytoin, azathioprine, and isoniazid) [8]. In our case, the PRCA diagnosis was made based on the Japanese PRCA guideline [9].…”

Section: Discussionmentioning

confidence: 99%

Successful treatment of a hemodialyzed patient with pure red cell aplasia associated with epoetin beta pegol therapy with cyclosporine

et al. 2015

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A 42-year-old man with end-stage renal failure had been receiving hemodialysis therapy since April 2009. Initially, darbepoetin alfa was administered to treat his renal anemia. After treatment was switched to epoetin beta pegol, the patient's hemoglobin levels rapidly decreased. He was diagnosed with pure red cell aplasia (PRCA) based on the results of a bone marrow examination. Epoetin beta pegol was strongly suspected to be the cause of the PRCA, and although he tested negative for anti-epoetin beta pegol antibodies, epoetin beta pegol was discontinued and cyclosporine therapy was initiated. Thereafter, his hemoglobin levels increased, and his anemia dramatically improved after 3 months.

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Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A. A nationwide cohort study in Japan for the PRCA Collaborative Study Group

Cited by 83 publications

References 21 publications

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

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

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

Successful treatment of a hemodialyzed patient with pure red cell aplasia associated with epoetin beta pegol therapy with cyclosporine

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