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Reduced intensity conditioning bone marrow transplantation for pure red cell aplasia: successful outcome but difficult post transplant course

Bone Marrow Transplantation volume 36, pages 81–82 (2005)Cite this article

We report a case of reduced intensity stem cell transplantation (RI-SCT) using cells from a matched unrelated donor, for the treatment of pure red cell aplasia (PRCA). Owing to graft failure, a second infusion of stem cells from the same donor yielded complete donor engraftment within 3 months from the initial transplant. PRCA is a rare haematological disease characterised by anaemia with absence of red-cell precursors in the bone marrow, but normal granulopoiesis and megakaryopoiesis.1 Acquired PRCA is associated with lymphoproliferative disorders, drugs, thymoma and autoimmune disorders. Infection, particularly human parvovirus B19, is associated with self-limited PRCA in healthy children and adults.2 It has been described after allogeneic bone marrow transplantation from ABO incompatible donors.3, 4 The pathogenic mechanisms of acquired PRCA generally relate to specific antibodies or direct T-cell lymphocyte toxicity to the red cell progenitors. Management of severe acquired or primary PRCA can be difficult. The mainstay of treatment includes immunosuppression with clinical responses of approximately 70%.5 More intensive approaches such as allogeneic stem cell transplantation (AlloSCT) are required for more severely affected patients, but TRM limits its application.6, 7 RI-SCT may confer benefits compared with conventional alloSCT8 and this possibly supports its use for the management of severe PRCA in younger patients where iron overload represents an ongoing clinical problem.

A 28-year-old female patient initially presented with reduced haemoglobin, normal leucocyte and platelet numbers. Autoimmune screen, liver function tests, B12 and folate were normal and parvovirus serology was negative. A diagnosis of PRCA was confirmed by bone marrow aspirate and trephine. Cytogenetic analysis confirmed normal female karyotype. Screening test for Fanconi's anaemia and paroxysmal nocturnal haemoglobinuria were negative. Radiological imaging ruled out thymoma. Treatment modalities attempted over 11 years included transfusion support, steroids, cyclosporin, low- and high-dose erythropoietin, azathioprine, fludarabine and granulocyte colony-stimulating factor (G-CSF).

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  1. Adult Leukaemia Unit, Manchester Christie Hospital, Wilmslow Road, Manchester, UK

    G Kochethu, H S Baden, E Jaworska, J Chang & R Chopra

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  1. G Kochethu
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Kochethu, G., Baden, H., Jaworska, E. et al. Reduced intensity conditioning bone marrow transplantation for pure red cell aplasia: successful outcome but difficult post transplant course. Bone Marrow Transplant 36, 81–82 (2005). https://doi.org/10.1038/sj.bmt.1704993

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