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Day 100 marrow glycophorin stain showing absence of erythroid precursors ( 400). (b) Day 240 marrow glycophorin stain demonstrating plentiful erythroblasts ( 400).
Source publication
Pure red cell aplasia (PRCA) occurred in the fourth month after an ABO-compatible nonmyeloablative allograft coincident with the cessation of immunosuppression and the onset of limited chronic GVHD. No secondary causes could be identified. Erythropoiesis was restored promptly and durably with the resumption of immunosuppression. A clonal T cell rec...
Contexts in source publication
Context 1
... was no history of blood loss. Red cell aplasia, confirmed by glycophorin immuno- histochemistry, was demonstrated on a day 100 marrow aspirate ( Figure 1a). No B cell lymphoma was demon- strated by morphology or flow cytometry. ...
Context 2
... day 138, the Hb had increased to 121 g/l with a modest reticulocytosis of 3%; this Hb has been maintained thereafter. The patient remains well with ongoing limited chronic GVHD, a normal Hb and normal marrow erythropoiesis (Figure 1b) at 8 months post transplant. A TCR g rearrangement was detectable in a peripheral blood sample at day 160, but was no longer detectable at 8 months. ...
Citations
... Several cases of PRCA were reported following allogenic HSCT, mostly in the situations of major ABO incompatibility between recipient and donor like our case, but it is also described in one case after ABO matched allogenic HSCT [4]. The use of a reducedintensity conditioning [5], sibling donors [6] or the absence of acute graft-versus host disease [6] are also identified as a risk factor of PRCA, In a review of literature, the Major ABO-incompatibility was the most common risk factor found in 120 patients, The roles of reduced intensity conditioning cells is controversial, and same for the use or not of Methotrexate in the prophylaxis of GVHD [7]. ...
... Management of PRCA after mismatched ABO incompatibility is not codified, a first option is a reduction of immunosuppression to enhance the graft-versus-plasma cell effects [3,4] but in our case it wasn't possible because of active chronic liver GVHD ,Other treatments are available but they are only evaluated in small numbers series. ...
Background: Pure Red Cell Aplasia (PRCA) is a rare complication of ABO mismatched hematopoietic stem cell transplantation; there isn’t no standard of care, here we report a case of successful treatment by Rituximab in a refractory PRCA and chronic graft versus host disease. Case Presentation: A 26-year-old woman with PRCA following ABOmismatched allogeneic HSCT for chronic myeloid leukemia, associated with steroid refractory chronic hepatic graft versus host disease, treated with 4 doses of Rituximab 375mg/m² weekly, with an increase in her hemoglobin level and improvement of her liver’s enzymes. Conclusion: The interest of this case is to report the important therapeutic result of Rituximab, widely used in literature, especially if chronic Graft Versus host disease is associated.
... For transplant recipients who have pure redcell aplasia caused by persistently elevated isohemagglutinins, the first therapeutic approach is the tapering of immunosuppressive agents to promote a graft-versus-plasma-cell effect. 4,17,18 In Shown are the hematocrit, reticulocyte count, anti-A antibody titer, red-cell transfusion requirement, and blood type before and after treatment first with rituximab and then with daratumumab (shading). The variation in the hematocrit after rituximab therapy reflects continued management with red-cell transfusions; the patient received a transfusion immediately before the initiation of daratumumab therapy. ...
Daratumumab, a human IgG1κ monoclonal antibody targeting CD38, is used to treat multiple myeloma. We describe successful treatment with daratumumab in a case of treatment-refractory pure red-cell aplasia after ABO-mismatched allogeneic stem-cell transplantation. The patient was a 72-year-old man with the myelodysplastic syndrome who received a transplant from an HLA-matched, unrelated donor with a major ABO incompatibility (blood group A in the donor and blood group O in the recipient). The patient had persistent circulating anti-A antibodies and no red-cell recovery 200 days after transplantation. Standard treatments had no effect. Within 1 week after the initiation of treatment with daratumumab, he no longer required transfusions.
Pure red cell aplasia (PRCA) and Evans syndrome following allogeneic hematopoietic stem cell transplantation (HSCT) from a blood type-matched donor are very rare. A 29-year-old Japanese woman with hematologic remission of Philadelphia chromosomepositive acute lymphoblastic leukemia underwent bone marrow transplantation from a blood type-matched, HLA 6/6 ― matched, unrelated donor in May 2008. Her clinical course after transplantation was favorable, and allowed for a gradual reduction in tacrolimus dosage. However, 12 months after transplantation, she developed PRCA related to allogeneic HSCT. The patientʼs PRCA was alleviated by stopping the dosage reduction for the immunosuppressive therapy and by continued administration of lowdose tacrolimus. Three months after the PRCA diagnosis, the patient developed Evans syndrome, so prednisolone (PSL) treatment was initiated. PSL treatment effectively treated the Evans syndrome, as was evident by the absence of cythemolysis two months later, and no relapse has occurred even after the PSL dose was reduced and then terminated. The fact that PRCA onset in this case occurred after blood type-matched allogeneic HSCT, during the late grafting phase, and in combination with Evans syndrome, suggests that the pathogenic mechanism may differ from that of previously reported cases of PRCA following blood type-incompatible allogeneic HSCT.
Extracorporeal photopheresis (ECP) has been used widely in the treatment of steroid-refractory chronic graft versus host disease (cGVHD). Several reports have applied an ‘early treatment’ approach due to the better response rates compared with late treatment. However, herein, we report a hematopoetic stem cell transplantation performed in a thalassemia major patient presenting with severe cGVHD who applied to our center for ECP treatment nearly 12 years after the onset of cGVHD.
Abstract Pure red cell aplasia (PRCA) is a haematological disorder characterised by normocytic anemia, reticulocytopenia, and selective erythroid hypoplasia with preservation of myelopoiesis and megakaryopoiesis. PRCA is most commonly idiopathic. It may also present secondary to a variety of conditions, such as parvovirus infection, drugs, autoimmune disorders, and hematological and solid malignancies. PRCA is a rare complication of lymphoproliferative disorders. Due to the limited reported cases of PRCA associated with lymphoid malignancies, the pathogenesis and optimal therapy remain undefined. There have been no systematic or randomized controlled trials regarding therapy, and the treatment choice is usually derived from single-arm studies of small numbers of patients. We report a patient with concurrent PRCA and Waldenstrom's Macroglobulinaemia (WM), in whom treatment of the underlying lymphoma resulted in complete remission of the PRCA. To the best of our knowledge, this is the first reported case of WM associated PRCA that was successfully treated with antilymphomatous chemotherapy.
Persistent anti-donor isoagglutinins after major ABO blood group incompatible hematopoietic stem cell transplantation may cause delayed red blood cell engraftment and post-transplant pure red cell aplasia.
We investigated the effect of pretransplant anti-donor isoagglutinin reduction by in vivo absorption and/or plasmapheresis on the incidence of pure red cell aplasia and the time to red blood cell engraftment in 153 hematopoietic stem cell transplant recipients with major ABO incompatibility.
Twelve patients (8%) developed pure red cell aplasia, 3/98 (3%) with, and 9/55 (16%) without prior isoagglutinin reduction (p=0.009). Red blood cell engraftment was faster in patients with isoagglutinin reduction; in addition, peripheral blood hematopoietic stem cell transplantation, acute graft-versus-host disease, and younger age were associated with faster red blood cell engraftment in Cox regression analysis. In patients with pure red cell aplasia the mean red blood cell engraftment occurred after 225 days (p<0.001) and was associated with a simultaneous decrease of anti-donor isoagglutinins. Patients with pure red cell aplasia had higher pretransplant anti-donor isoagglutinin titers (p=0.001) and received more post-transplant red blood cell transfusions (p<0.001).
Following major ABO incompatible hematopoietic stem cell transplantation, pure red cell aplasia and delayed red blood cell engraftment depend on the levels of anti-donor isoagglutinins and are efficiently prevented by the pretransplant removal of these isoagglutinins. The benefits of reducing the time of transfusion-dependency and transfusion-associated risks must be carefully balanced against the potential side effects of isoagglutinin reduction.
Peripheral destruction of sickled erythrocytes is a cardinal feature of sickle cell disease (SCD). Less well established is the potential contribution of ineffective erythropoiesis to the pathophysiology of this hemoglobinopathy. Since patients with SCD frequently develop mixed hematopoietic chimerism after allogeneic nonmyeloablative stem cell transplantation, we used this opportunity to directly compare the differentiation and survival of SCD and donor-derived erythropoiesis in vivo. Donor and recipient erythropoiesis was compared in 4 patients with SCD and 4 without SCD who developed stable mixed hematopoietic chimerism following transplant. Molecular analysis of chimerism in peripheral blood and bone marrow demonstrated higher expression of donor-derived beta-globin RNA relative to the level of donor-derived genomic DNA in patients with SCD. Analysis of chimerism in immature (glycophorin A-positive [GYPA(+)], CD71(hi)) and mature (GYPA(+), CD71(neg)) erythroblasts confirmed the intramedullary loss of SS erythroblasts with progressive maturation. In patients with SCD, relative enrichment of donor erythroid precursors began to appear at the onset of hemoglobinization. Ineffective erythropoiesis of homozygous hemoglobin S (SS) progenitors thus provides a maturation advantage for homozygous hemoglobin A (AA) or heterozygous hemoglobin S/hemoglobin A (SA) donor erythroid precursor cells that results in greater donor contribution to overall erythropoiesis following stem-cell transplantation and improvement of clinical disease.
Bone Marrow Transplantation is a high quality, peer-reviewed journal covering all aspects of clinical and basic haemopoietic stem cell transplantation.
