Skip to main content

Advertisement

SpringerLink
Log in

Pure red-cell aplasia following major and bi-directional ABO-incompatible allogeneic stem-cell transplantation: recovery of donor-derived erythropoiesis after long-term treatment using different therapeutic strategies

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Blood group incompatibility between donor and recipient of allogeneic stem cell transplants may be associated with post-transplant erythroid aplasia. A total of 548 patients (pts) received allogeneic transplant for malignant and non-malignant hematologic disorders. In a retrospective analysis, the prevalence and outcome of pure red-cell aplasia (PRCA) in 44 pts with major and bi-directional ABO-mismatch were investigated. Bone marrow grafts were major ABO incompatible in 30 pts; there was bi-directional mismatch in the remaining 14 pts. The median number of transplanted mononuclear cells (NC) was 4.74 × 108/kg (range 0.1–26.4) including CD34+ cells, 3.02 × 106/kg (range 0.9–21.7). Granulocyte engraftment >0.5 × 10e9/l occurred after a median of 21 days (7–32), and platelet exceeded >50 × 10e9/l after a median of 23.5 days (12–109). Acute and chronic graft vs host disease (GVHD) developed in 23 (52%) and 26 (59%) of the patients, respectively. Six (13%) patients transplanted with major and bi-directional ABO-incompatibility developed PRCA. The treatment of PRCA consisted of plasmapheresis (PEX), rapid cyclosporine (CsA) discontinuation, donor lymphocyte infusions (DLI), erythropoietin (EPO), azathioprine, and rituximab. The therapy resulted in erythroid recovery in five out of six patients after a median of 13 months (range 3–16). The median number of transfused red blood cells (RBCs) was 36 U (range 8–57). With a median follow-up of 37 months, the 5-year probability of overall survival (OS) for the PRCA group was 66%. Major ABO mismatch may lead to delayed donor erythroid engraftment. It results in long-term transfusion dependence and, therefore, the risk of iron overload. The therapy is long lasting, but usually effective in majority of patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Bavaro P, Di Girolamo G, Olioso P, Papalinetti G, Iacone A, Accorsi P, Di Bartolomeo (1999) Donor lymphocyte infusion as therapy for pure red cell aplasia following bone marrow transplantation. Br J Haematol 104(4):930–931

    Article  PubMed  CAS  Google Scholar 

  2. Bensinger WI, Buckner CD, Thomas ED, Clift RA (1982) ABO-incompatible marrow transplants. Transplantation 33:427–429

    Article  PubMed  CAS  Google Scholar 

  3. Bolan CD, Leitman SF, Griffith LM, Wesley RA, Procter JL, Stroncek DF, Barrett J, Childs RW (2001) Delayed donor red cell chimerism and pure red cell aplasia following major ABO-incompatible nonmyeloablative hematopoietic stem cell transplantation. Blood 98:1687–1694

    Article  PubMed  CAS  Google Scholar 

  4. Deotare UR, Vishwabandya A, Mathews V, George B, Srivastava A, Chandy M (2006) Response to high-dose dexamethasone for acquired pure red cell aplasia following ABO-mismatched allogeneic stem cell transplantation. Bone Marrow Transplant 37:1149–1150

    Article  PubMed  CAS  Google Scholar 

  5. Gmur J, Burger J, Schaffner A, Neftel K, Oelz O, Frey D, Metaxas M (1990) Pure red cell aplasia of long duration complicating major ABO-incompatible bone marrow transplantation. Blood 75:290–295

    PubMed  CAS  Google Scholar 

  6. Griffith LM, McCoy JP Jr, Bolan CD, Stroncek DF, Pickett AC, Linton GF, Lundqvist A, Srinivasan R, Leitman SF, Childs RW (2005) Persistence of recipient plasma cells and anti-donor isohaemagglutinins in patients with delayed donor erythropoiesis after major ABO incompatible non-myeloablative haematopoietic cell transplantation. Br J Haematol 128:668–675

    Article  PubMed  CAS  Google Scholar 

  7. Helbig G, Stella-Holowiecka B, Krawczyk-Kulis M, Wojnar J, Markiewicz M, Wojciechowska-Sadus M, Kopera M, Kruzel T, Najda J, Nowak K, Holowiecki J (2005) Successful treatment of pure red cell aplasia with repeated, low doses of rituximab in 2 patients after ABO-incompatible allogeneic haematopoietic stem cell transplantation for acute myeloid leukaemia. Haematologica 90(Suppl):ECR33

    PubMed  Google Scholar 

  8. Hows JM, Chipping PM, Palmer S, Gordon-Smith EC (1983) Regeneration of peripheral blood cells following ABO incompatible allogeneic bone marrow transplantation for severe aplastic anaemia. Br J Haematol 53:145–151

    PubMed  CAS  Google Scholar 

  9. Hyell A, Aul C, Runde V, Arning M, Schneider W, Wernet P (1991) Treatment of pure red cell aplasia after major ABO-incompatible bone marrow transplantation with recombinant erythropoietin. Blood 77:906

    Google Scholar 

  10. Lee JH, Lee KH, Kim S, Lee JS, Kim SH, Kwon SW, Kim WK (2000) Anti-A isoagglutinin as a risk factor for the development of pure red cell aplasia after major ABO-incompatible allogeneic bone marrow transplantation. Bone Marrow Transplant 25:179–184

    Article  PubMed  CAS  Google Scholar 

  11. Lee JH, Lee JH, Choi SJ, Kim S, Soel M, Kwon SW, Park CJ, Chi HS, Lee JS, Kim WK, Lee KH (2003) Changes of isoagglutinin titres after ABO-incompatible allogeneic stem cell transplantation. Br J Haematol 120:702–710

    Article  PubMed  CAS  Google Scholar 

  12. Maschan AA, Skorobogatova EV, Balsashov DN, Pashanov ED, Trakhtman PE, Schipitizina IP, Skvortsova YV, Rumiantzev AG (2002) Successful treatment of pure red cell aplasia with a single dose of rituximab in a child after major ABO incompatible peripheral allogeneic stem cell transplantation for acquired aplastic anemia. Bone Marrow Transplant 30:405–407

    Article  PubMed  CAS  Google Scholar 

  13. Mehta J, Powles R, Horton C, Milan S, Singhal S, Treleaven J (1995) Relationship between donor-recipient blood group incompatibility and serum bilirubin after allogeneic bone marrow transplantation from HLA-identical siblings. Bone Marrow Transplant 15:853–858

    PubMed  CAS  Google Scholar 

  14. Mielcarek M, Leisenring W, Torok-Storb B, Storb R (2000) Graft-versus-host disease and donor-directed hemagglutinin titrem after ABO-mismatched related and unrelated marrow allografts: evidence for graft -versus-plasma cell effect. Blood 96:1150–1156

    PubMed  CAS  Google Scholar 

  15. Or R, Naparstek E, Mani N, Slavin S (1991) Treatment of pure red cell aplasia following ABO-incompatible bone marrow transplantation: two case reports with successful response to plasmapheresis. Transpl Int 4:99–102

    Article  PubMed  CAS  Google Scholar 

  16. Paltiel O, Cournoyer D, Rybka W (1993) Pure red cell aplasia following ABO-incompatible bone marrow transplantation: response to erythropoietin. Transfusion 33:418–421

    Article  PubMed  CAS  Google Scholar 

  17. Pegs KS, Morris EC, Kottaridis PD, Geary J, Goldstone AH, Linch DC, Mackinnon S (2002) Outcome of major ABO-incompatible nonmyeloablative hematopoietic stem cell transplantation may be influenced by conditioning regimen. Blood 99:4642–4644

    Article  Google Scholar 

  18. Rabitsch W, Knobl P, Prinz E, Keil F, Greinix H, Kahls P, Worel N, Jansen M, Horl WH, Derfler K (2003) Prolonged red cell aplasia after major ABO-incompatible allogeneic hematopoietic stem cell transplantation: removal of persisting isohemagglutinins with Ig-Therasorb® immunoadsorption. Bone Marrow Transplant 32:1015–1019

    Article  PubMed  CAS  Google Scholar 

  19. Santamaria A, Sureda A, Martino R, Domingo-Albos A, Muniz-Diaz E, Brunet S (1997) Successful treatment of pure red cell aplasia after major ABO-incompatible T cell-depleted bone marrow transplantation with erythropoietin. Bone Marrow Transplant 12:1105–1107

    Article  Google Scholar 

  20. Schetelig J, Breitschaft A, Kroger N, Zabelina T, Ebell W, Bornhauser M, Haack A, Ehninger G, Salama A, Siegert W (2005) After major ABO-mismatched allogeneic hematopoietic progenitor stem cell transplantation, erythroid engraftment occurs later in patients with donor blood group A than donor blood group B. Transfusion 45:779

    Article  PubMed  Google Scholar 

  21. Seebach JD, Stussi G, Passweg JR, Loberiza FR Jr, Gajewski JL, Keating A, Goerner M, Rowlings PA, Tiberghien P, Elfenbein GJ, Gale RP, van Rood JJ, Reddy V, Gluckman E, Bolwell BJ, Klumpp TR, Horowitz MM, Ringden O, Barret AJ (2005) GVHD Working Committee of Center for International Blood and Marrow Transplant Research. ABO blood group barrier in allogeneic bone marrow transplantation revisited. Biol Blood Marrow Transplant 11:1006–1013

    Article  PubMed  Google Scholar 

  22. Sora F, De Matteis S, Piccirillo N, Chiusolo P, Laurenti L, Putzulu R, Leone G, Sica S (2005) Rituximab for pure red cell aplasia after ABO-mismatched allogeneic peripheral blood progenitor cell transplantation. Transfusion 45:643–644

    Article  PubMed  Google Scholar 

  23. Verholen F, Stadler M, Helg C, Chalandon Y (2004) Resistant pure red cell aplasia after allogeneic stem cell transplantation with major ABO mismatch treated by escalating dose donor leukocyte infusion. Eur J Haematol 73:441–446

    Article  PubMed  CAS  Google Scholar 

  24. Worel N, Greinix HT, Schneider B, Kurz M, Rabitsch W, Knobl P, Reiter E, Derfler K, Fischer G, Hintenberger W, Hocker P, Kahls P (2000) Regeneration of erythropoiesis after related- and unrelated-donor BMT or peripheral blood HPC transplantation: a major ABO mismatch means problems. Transfusion 40:543–550

    Article  PubMed  CAS  Google Scholar 

  25. Volin L, Ruutu T (1990) Pure red-cell aplasia of long duration after major ABO-incompatible bone marrow transplantation. Acta Haematol 84:195–197

    Article  Google Scholar 

  26. Yang MH, Hsu HC (2001) Pure red cell aplasia after ABO-incompatible allogeneic stem cell transplantation in severe aplastic anaemia with response to steroids: a case report and literature review. Ann Hematol 80:299–301

    Article  PubMed  CAS  Google Scholar 

  27. Zhu K, Chen J, Chen S (2005) Treatment of Epstein–Barr virus-associated lymphoproliferative disorder (EBV–PTLD) and pure red cell aplasia (PRCA) with rituximab following unrelated cord blood transplantation: a case report and literature review. Hematology 10:365–370

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Haematology and Bone Marrow Transplantation, Silesian Medical University, Reymont Street 8, 40-027, Katowice, Poland

    Grzegorz Helbig, Beata Stella-Holowiecka, Jerzy Wojnar, Malgorzata Krawczyk, Slawomira Krzemien, Maria Wojciechowska-Sadus, Mirosław Markiewicz, Iwona Wylezol, Malgorzata Kopera & Jerzy Holowiecki

Authors
  1. Grzegorz Helbig
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Beata Stella-Holowiecka
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jerzy Wojnar
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Malgorzata Krawczyk
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Slawomira Krzemien
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Maria Wojciechowska-Sadus
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Mirosław Markiewicz
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Iwona Wylezol
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Malgorzata Kopera
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Jerzy Holowiecki
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Grzegorz Helbig.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Helbig, G., Stella-Holowiecka, B., Wojnar, J. et al. Pure red-cell aplasia following major and bi-directional ABO-incompatible allogeneic stem-cell transplantation: recovery of donor-derived erythropoiesis after long-term treatment using different therapeutic strategies. Ann Hematol 86, 677–683 (2007). https://doi.org/10.1007/s00277-007-0304-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-007-0304-8

Keywords

Search

Navigation