Long‐term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following immunosuppressive therapy: a final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group

Abstract

SummaryImmunosuppressive therapy has been employed as the initial treatment for acquired chronic pure red cell aplasia (PRCA), such as idiopathic, thymoma-associated, or large granular lymphocyte (LGL) leukaemia-associated PRCA, which is thought to be immune-mediated. To explore the overall long-term outcome following immunosuppression and to identify the risk factors for death in these disorders, we conducted nationwide surveys in Japan 2004 and 2006, and identified a total of 185 patients with acquired chronic PRCA, including 72 idiopathic, 41 thymoma-associated and 14LGL leukaemia-associated cases of PRCA for whom data was available. The present study evaluated 127 patients with these three subsets of PRCA. The median overall survival has not yet been reached in idiopathic PRCA. The estimated median overall survival times in patients with thymomaassociated and LGL leukaemia-associated PRCA were 142Á1 and 147Á8 months, respectively. Twenty-two deaths were reported, and the response to induction therapy and relapse of anaemia were found to be associated with death. The major causes of death were infection in seven patients and organ failure in another seven patients. The results suggest that maintenance therapy and the management of infectious complications are crucial for improving the prognosis of chronic PRCA.

Thymic Epithelial Tumor-Associated Cytopenia: A 10-Year Observational Study in France

“…This has already been reported (for cases of PRCA) by the Japanese collaborative group and also seems to be true for our immune thrombocytopenia patients. 20 The present study had several limitations. First, the study's retrospective design probably increased the proportion of missing data.…”

Invasive Thymoma with Pure Red Cell Aplasia and Amegakaryocytic Thrombocytopenia

“…1-3 Immunosuppressive therapy is the most effective treatment for PRCA, while total thymectomy has been reported to be less effective. 2 …”

Reversal of pure red cell aplasia by varicella zoster virus infection

“…Published: May 03, 2019 3/10 blood transfusions as needed to more speci ic measures such as allogeneic hematopoietic stem cell transplantation (HSCT) as illustrated in table 2 [1][2][3][4][5][16][17][18][19][20][33][34][35][36][37][38]. The course and prognosis of PRCA vary depending on being hereditary or acquired and they depend on the underlying cause to a large extent [39][40][41][42][43]. However, most patients with chronic PRCA can be successfully treated with immunosuppressive therapies, but they require maintenance treatment with immunosuppressive agents [39][40][41][42][43].…”