Pure red cell aplasia after hematopoietic stem cell transplantation - experimental therapeutic approaches

Vo, Anh Khoi; Hervig, Tor; Reikvam, Håkon

doi:10.1080/13543784.2022.2113055

Cited by 2 publications

(4 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The purpose of this retrospective analysis was to identify possible risk factors for PRCA occurrence among patients transplanted across AB0 mismatch and to analyze different treatments applied to PRCA cases with relative responses. The incidence of PRCA at day 60 in our cohort was 13.4%, in line with other reports ( 3 , 4 , 6 , 7 ). The sole variable resulting from multivariate analysis as predictive for PRCA developed was pre-HSCT IgG IHA, with an optical cut-off value of 1/64 (100% sensitivity and 68.8% specificity).…”

Section: Discussionsupporting

confidence: 93%

“…An indispensable element in transplant planning is the selection of a suitable donor in terms of human leukocyte antigen (HLA) match, while ABO compatibility is not mandatory ( 2 ). However, the ABO mismatch between recipient and donor must be taken into account as it may lead to acquired pure red cell aplasia (PRCA) ( 3 , 4 ), with prolonged transfusion requirement and the risk of iron overload. Up to 50% of HSCTs are performed across ABO incompatibility but the influence of this condition on post-transplant outcomes remains controversial ( 3 – 5 ).…”

Section: Introductionmentioning

confidence: 99%

“…Up to 50% of HSCTs are performed across ABO incompatibility but the influence of this condition on post-transplant outcomes remains controversial ( 3 – 5 ). The incidence of PRCA after AB0 mismatched transplant varies from 10 to 29% of cases ( 3 , 4 , 6 , 7 ), and it is observed after major or bi-directional AB0 mismatch. In major AB0 mismatch, accounting for 20–25% of transplants, the recipient showed anti-donor AB0 antibodies, as for a group 0 patient receiving cells from a group A or B donor.…”

Section: Introductionmentioning

confidence: 99%

“…However, the ABO mismatch between recipient and donor must be taken into account as it may lead to acquired pure red cell aplasia (PRCA) ( 3 , 4 ), with prolonged transfusion requirement and the risk of iron overload. Up to 50% of HSCTs are performed across ABO incompatibility but the influence of this condition on post-transplant outcomes remains controversial ( 3 – 5 ). The incidence of PRCA after AB0 mismatched transplant varies from 10 to 29% of cases ( 3 , 4 , 6 , 7 ), and it is observed after major or bi-directional AB0 mismatch.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Pure red cell aplasia among ABO mismatched hematopoietic stem cell transplant recipients: a 13-years retrospective study and literature review

Metafuni,

Busnego Barreto,

Valentini

et al. 2024

Front. Oncol.

View full text Add to dashboard Cite

BackgroundPure red cell aplasia (PRCA) is a possible complication after allogeneic hematopoietic stem cell transplantation (HSCT) with major ABO incompatibility. Patients experience delayed engraftment of the erythroid series, with prolonged transfusion-dependent anemia and iron overload.MethodsWe performed a revision of the most recent literature about post-HSCT PRCA treatment procedures. Moreover, we conducted a retrospective study, over the last 13-years, which included all consecutive major ABO mismatched HSCT performed in our unit, with the aim to assess PRCA incidence, risk factors, and response to different treatments. Overall, 194 patients received a major ABO mismatched transplant from 2010 to 2022. For each patient, data about demographic and transplant characteristics, engraftment, blood transfusion, and possible treatment received were collected.ResultsThe literature review returned 23 eligible papers on PRCA treatment, with high success rate using plasma-exchange (PEX) and immunoadsorption procedures, daratumumab, and eltrombopag. Our study identified a total of 24 cases of PRCA. Among risk factors for PRCA development, we have found older recipient age (p=0.01), high pre-HSCT IgG and IgM IHA titer (p<0.0001), major rather than bidirectional ABO incompatibility (p=0.02), low T CD8 lymphocyte count in the graft (p=0.006), relative donor (p=0.02) and bone marrow as stem cell source (p=0.002). However, multivariate analysis confirmed only pre-HSCT IgG IHA titer as the unique risk factor for PRCA occurrence. The optimal cut-off value of pre-HSCT IgG IHA for PRCA development, resulted to be 1/64, with a 100% sensitivity and 68.8% specificity (p<0.0001). All patients with PRCA had received rhEPO and transfusion support and 20 patients received additional treatments like PEX, rituximab, and more recently daratumumab. Comprehensively, PEX and rituximab obtained a response in half of the cases, at a variable time, while the few cases of patients we treated with daratumumab suggest promising results. The overall response rate in our cohort was 75%, with significantly better survival (94.4% vs. 16.7%) and lower transplant-related mortality (6.3% vs. 80%) for PRCA responders.ConclusionsStandardized guidelines on when and how to treat PRCA are necessary because the current treatment is controversial among centers.

show abstract