Successful treatment of pure red cell aplasia with high-dose dexamethasone after ABO-incompatible allogeneic hematopoietic stem cell transplantation

Abstract

The literature reports an incidence of Pure Red Cell Aplasia (PRCA) ranging from 6-30% of all cases of ABO-incompatible HSCT. Although most patients resolve spontaneously after withdrawal immunosuppression, some of them require more aggressive treatment to manage this condition.

Successful treatment pure red cell aplasia after ABO major mismatched allogeneic hematopoietic stem cell transplantation with avatrombopag and low dose rituximab

“…The incidence of PRCA after ABO major and bidirectional mismatched allo‐HSCT was 6.9% in our center, similar to that reported in the literature 16,17 . The presence of anti‐A IHA prior to transplantation, reduced intensity conditioning, absence of aGVHD, sibling donors, and CsA as GVHD prophylaxis are all risk factors for developing PRCA 12 . In our study, all donor and recipient blood groups were A + and O + in the five patients with PRCA, and only one patient with PRCA received myeloablative conditioning.…”

“…16,17 The presence of anti-A IHA prior to transplantation, reduced intensity conditioning, absence of aGVHD, sibling donors, and CsA as GVHD prophylaxis are all risk factors for developing PRCA. 12 In our study, all donor and recipient blood groups were A + and O + in the five patients with PRCA, and only one patient with PRCA received myeloablative conditioning. Major ABO incompatible allo-HSCT is characterized by the presence of preformed antidonor IHA, particularly when the donor blood type is A.…”

“…20 According to additional research, the median time for untreated PRCA patients to achieve remission was 133-229 days. 12 Repeated transfusions can lead to iron overload and poor quality of life. Some patients still need various treatments.…”

“…The patients may benefit from various approaches including high-dose steroids, eltrombopag, erythropoietin (EPO), plasma exchange (PE), immunoadsorption, donor lymphocyte infusion (DLI), treatment with rituximab, bortezomib, or daratumumab, and tapering or discontinuation of immunosuppression. 2,[7][8][9][10][11][12][13][14] Yet, the best therapeutic strategy for PRCA still remains unclear. In this article, we documented a retrospective, observational study treating five patients with PRCA following major ABO-incompatible allo-HSCT with avatrombopag and lower doses of rituximab.…”

“…This prolonged dependency on transfusions resulted in persistent fatigue, iron overload, and a diminished quality of life until PRCA remission was achieved. The patients may benefit from various approaches including high‐dose steroids, eltrombopag, erythropoietin (EPO), plasma exchange (PE), immunoadsorption, donor lymphocyte infusion (DLI), treatment with rituximab, bortezomib, or daratumumab, and tapering or discontinuation of immunosuppression 2,7–14 . Yet, the best therapeutic strategy for PRCA still remains unclear.…”