Pure red cell aplasia of long duration complicating major ABO- incompatible bone marrow transplantation [see comments]

Abstract

In 3 of 15 consecutive patients receiving a human leukocyte antigen (HLA)-identical but major ABO incompatible bone marrow transplant (BMT), pure red cell aplasia (PRA) lasting 5 to 8 months was observed. Titers of the incompatible anti-A agglutinin before infusion of the red blood cell (RBC)-depleted BMT was very high in one, and in the usual range in two patients. Decrease of agglutinin titers during the first 4 weeks after BMT were comparable between PRA patients and those of ABO- incompatible BMT recipients with timely RBC recovery. However, in PRA patients, agglutinin titers rose again and remained elevated for 19 to 28 weeks. RBC engraftment and reticulocyte recovery ultimately occurred spontaneously and coincided with the decrease of agglutinin titers below 16. These observations indicate that PRA is antibody-dependent in this setting. Furthermore, it is conceivable that cyclosporine facilitates recipient-derived antibody synthesis after major ABO- incompatible BMT.

Successful treatment of pure red cell aplasia because of ABO major mismatched stem cell transplant

“…ABO major mismatched transplants have been welldocumented to result in a delay in engraftment of the erythroid cell line. 1,2 Delayed erythrocyte engraftment is thought to be a result of elevated, persistent isohemagglutinins that are formed against the donor progenitor red blood cells. ABO mismatched stem cell transplants are routinely performed at our institution, and the preparation for this includes a red cell depletion with hydroxyl ethyl starch performed on only stem cells from a bone marrow source.…”

“…Pure red cell aplasia (PRCA) is a known complication of ABO major mismatched allogeneic hematopoietic stem cell transplantation (HCT). 1,2 An ABO major mismatched transplant occurs when a HCT recipient can make isohemagglutinins against the donor product. This occurs in recipients who are ABO type A, receiving an AB or B transplant, type B receiving a type A or AB transplant, or type O receiving an A, B, or AB transplant.…”

“…Treatment of an immunocompromised patient with PRCA resulting from a parvovirus infection includes removal of immunosuppression (if possible), and/or IVIg; however, the PRCA because of parvovirus also may, in some cases, be self-limiting. 1,17,18 Parvovirus is transmitted via respiratory secretions, or in blood products such as plasma, red blood cells or platelets, or IVIg. [19][20][21][22][23] Patients who are undergoing a stem cell transplant are not only immunocompromised, but are also typically receiving multiple blood products, which are a known source of parvovirus transmission.…”

Efficacy of plasmapheresis for the treatment of pure red blood cell aplasia after allogeneic stem cell transplantation

“…P ure red blood cell aplasia (PRCA) occurs in 6% to 30% of ABO major-incompatible hematopoietic stem cell transplantations (HSCTs), [1][2][3][4][5] and in some cases it may resolve spontaneously, but usually it requires therapy to avoid multiple red blood cell (RBC) transfusions, iron overload, and increased risk of infections. The mechanism of PRCA after ABOincompatible HSCT is still unclear, but the persistence of memory B lymphocytes of recipient origin able to produce hemagglutinins against ABO antigens on donor RBCs may play a role in the pathogenesis.…”

Frequency and prognostic value of D alloantibodies after D‐mismatched allogeneic hematopoietic stem cell transplantation after reduced‐intensity conditioning

“…[2][3][4] Reports on transplant-associated problems after ABO-incompatible HSCT following reducedintensity conditioning (RIC) consisted of occurrence of pure red blood cell (RBC) aplasia due to persistence of host-derived isohemagglutinins or delayed immune hemolysis due to rapid isohemagglutinin production of donor lymphocytes. [5][6][7][8] Besides the ABO blood group system, Rh antigens, especially the D antigen are clinically the most significant factors for alloimmunization that usually occur after exposure of D-individuals to D+ blood components. 9,10 The risk for anti-D alloimmunization in D-volunteers given D+ RBCs is higher than 80% and up to 20% of D-women developed an anti-D during pregnancy before the routine use of anti-D prophylaxis.…”