Pure Red-Cell Aplasia of Long Duration after Major ABO-Incompatible Bone Marrow Transplantation

Abstract

We describe a patient with an exceptionally long-lasting red-cell aplasia of 330 days following ABO-incompatible bone marrow transplantation (BMT). Before BMT, the anti-B titre was high, 1:1,024, and it was only temporarily reduced by extensive plasma exchange. The anti-B titre remained above the level of 1:64 for 270 days, and host-derived isoagglutinin could still be detected 3 years after BMT. In vitro bone marrow cultures during the red-cell aplasia showed greatly reduced numbers or total absence of CFU-E, while the number of BFU-E colonies was only moderately subnormal. Six years after BMT, bone marrow and peripheral-blood cell counts are normal.

Prevention of pure red cell aplasia after major or bidirectional ABO blood group incompatible hematopoietic stem cell transplantation by pretransplant reduction of host anti-donor isoagglutinins

“…[13][14][15] In the few studies in which PRCA occurred despite isoagglutinin reduction, a rebound of anti-donor isoagglutinins suggests post-transplant transfusion of recipient-type RBC. 16 If the isoagglutinins are only temporarily removed prior to the HSCT, they may rebound soon after transplantation to an even higher level. 17 In contrast, pretransplant anti-donor isoagglutinin reduction by the above mentioned strategies in combination with post-transplant transfusion of donor-type RBC may provide a durable suppression of anti-donor isoagglutinins and facilitate RBC engraftment.…”

“…25 Major ABO-incompatibility was the most common risk factor described in 120 patients, bidirectional ABO incompatibility was found in 5 patients. 16,25,37 Two of the remaining three patients developed PRCA after ABO compatible HSCT 41,49 and one after autologous HSCT. 39 No clear explanation was found for the PRCA in these patients.…”

“…Neutrophil engraftment was reached after 17 days (95%-CI [16][17][18][19] and platelet engraftment after 32 days (95%-CI 27-38) without differences between the groups (p=0.379 and p=0.364, respectively). As shown in Figure 1B, patients who underwent anti-donor isoagglutinin reduction had faster RBC engraftment (p<0.001).…”

“…The only two risk factors identified were a lack of pretransplant isoagglutinin reduction and the blood group constellation A or AB donor into an O group recipient. A review of the published literature identified 128 patients with PRCA in 18 case series [11][12][13]15,[20][21][22][23][24][25][26][27][28][29][30][31][32][33] and 35 case reports 16,18,19, with an overall incidence of 15% (range, 2-50%). 12,22 However, this may overestimate the true incidence due to a publication bias.…”

ABO-incompatible bone marrow transplantation: a GITMO survey of current practice in Italy and comparison with the literature

“…IHAs, even at low titre, can be responsible for delayed erythropoiesis in transplanted patients with major ABO incompatibility and also for a number of cases of pure red cell aplasia. [80][81][82][83][84][85] Furthermore, the cases of alloimmune haemolysis due to incompatibilities involving non-ABO RBC antigens emphasize the importance of close immunohaematological monitoring of patients undergoing HSCT even in the absence of ABO/Rh mismatch. 42 We believe that in cases of major ABO incompatibility, it is advisable to monitor recipient IHA titres on a weekly basis, especially when pretransplant levels are high.…”