Abstract
Autoimmune cytopenias are rare but serious complications after hematopoietic stem cell transplantation (HSCT). We performed splenectomy in 2 patients who had severe autoimmune cytopenias after allogeneic HSCT (allo-HSCT) that were resistant to immunosuppressive treatment. The first patient underwent unrelated allo-HSCT for chronic granulocytic leukemia (CGL) in July 2000. Seven months later, red blood cell and platelet counts went down. The results of a direct Coombs test were intermittently positive. The patient was resistant to therapy with steroids and high-dose immunoglobulin. After a splenectomy was performed in February 2001, the hemoglobin concentration and platelet count improved. Her blood counts remained stable, with a hemoglobin level of approximately 110 g/L and a platelet count >100 x 109/L. She continued therapy with itraconazole, valacyclovir, and penicillin. Some months later, the patient was readmitted for fulminant septic infection, which had a fatal outcome. The second patient underwent related allo-SCT for CGL in January 2003. Seven months later, he was readmitted for intraocular bleeding accompanied by severe thrombocytopenia with antiplatelet antibodies. The patient was resistant to steroid and high-dose immunoglobulin therapy. A splenectomy was performed in September 2003. His platelet count normalized and remains stable. The patient continues therapy with itraconazole, valacyclovir, and penicillin and has not experienced any serious infection. We assume that splenectomy is an effective treatment for resistant immune cytopenias after allo-HSCT. However, severe late infections can compromise the outcome.
Similar content being viewed by others
References
Deeg JH. Delayed complications after hematopoietic cell transplantation. In: Thomas ED, Blume KG, Forman SJ, eds.Hematopoietic Cell Transplantation. Oxford, UK: Blackwell Science; 1999:776–788.
Roberts MM, To LB, Gillis D, et al. Immune reconstitution following peripheral blood stem cell transplantation, autologous bone marrow transplantation and allogeneic bone marrow transplantation.Bone Marrow Transplant. 1993;12:469–475.
Atkinson K. Reconstruction of the hemopoietic and immune systems after marrow transplantation.Bone Marrow Transplant. 1990;5:209–226.
Sherer Y, Shoenfeld Y. Autoimmune diseases and autoimmunity post bone-marrow transplantation.Bone Marrow Transplant. 1998;22:873–881.
Itoh M, Takahashi T, Sakaguchi N, et al. Thymus and autoimmunity: production of CD25+CD4+ naturally anergic and suppressive T cells as a key function of the thymus in maintaining immunologic self-tolerance.J Immunol. 1999;162:5317–5326.
Lambertenghi-Deliliers GL, Annaloro C, Della Volpe A, Oriani A, Pozzoli E, Soligo D. Multiple autoimmune events after autologous bone marrow transplantation.Bone Marrow Transplant. 1997;19:745–747.
Ishikawa F, Shigematsu H, Gondo H, et al. Autoreactive antibodies following autologous peripheral blood stem cell transplantation.Bone Marrow Transplant. 1998;22:729–731.
Kottaridis PD, Rees H, Smith G, et al. A fatal case of autoimmune thrombocytopenia with an IgM anti-GPIb/IX following one antigen mismatched unrelated donor bone marrow transplantation.Bone Marrow Transplant. 1999;23:739–741.
Ting SS, Ziegler JB, Vowels MR. Acquired autoimmune thrombocytopenia post-bone marrow transplantation for severe combined immunodeficiency.Bone Marrow Transplant. 1998;21:841–843.
Lee SJ, Churchill WH, Konugres A, et al. Idiopathic thrombocytopenic purpura following allogeneic bone marrow transplantation treated with anti-D immunoglobulin.Bone Marrow Transplant. 1997;19:173–174.
Klumpp TR, Herman JH, Macdonald JS, Schnell MK, Mullaney M, Man KF. Autoimmune neutropenia following peripheral blood stem cell transplantation.Am J Hematol. 1992;41:215–217.
Hequet O, Salles G, Ketterer N, et al. Autoimmune thrombocytopenic purpura after autologous stem cell transplantation.Bone Marrow Transplant. 2003;32:89–95.
Ashihara E, Shimazadi C, Hirata T, et al. Autoimmune thrombocytopenia following peripheral blood stem cell autografting.Bone Marrow Transplant. 1993;12:297–299.
Ketterer N, Sonet A, Dumontet C, et al. Toxicities after peripheral progenitor cell transplantation for lymphoid malignancies: analysis of 300 cases in a single institution.Bone Marrow Transplant. 1999;23:1309–1315.
Sivakumaran M, Hutchinson RM, Pringle H, et al. Thrombocytopenia following autologous bone marrow transplantation: evidence for autoimmune aetiology and B cell clonal involvement.Bone Marrow Transplant. 1995;15:531–536.
Drobyski WR, Potluri J, Sauer D, Gottschall JL. Autoimmune hemolytic anemia following T-cell-depleted allogeneic bone marrow transplantation.Bone Marrow Transplant. 1996;17:1093–1099.
Jillella AP, Kallab AM, Kutlar A. Autoimmune thrombocytopenia following autologous hematopoietic cell transplantation: review of literature and treatment options.Bone Marrow Transplant. 2000;26:925–927.
Hartert A, Willenbacher W, Günzelmann S, et al. Successful treatment of thrombocytopenia and hemolytic anemia with IvIG in a patient with lupus-like syndrome after mismatched related PBSCT.Bone Marrow Transplant. 2001;27:337–340.
Tosi P, Bandini G, Tazzari P, et al. Autoimmune neutropenia after unrelated bone marrow transplantation.Bone Marrow Transplant. 1994;14:1003–1004.
De Lord C, Marsh JC, Smith JG, Singer CR, Gordon-Smith EC. Fatal autoimmune pancytopenia following bone marrow transplantation for aplastic anaemia.Bone Marrow Transplant. 1996;18:237–239.
Damoiseaux JG, van Breda Vriesman PJ. Pathogenesis of cyclosporin A-induced autoimmunity: absence of T-cell reactivity towards syngeneic antigen presenting cells.Autoimmunity. 1998;27:45–56.
Bashey A, Owen I, Lucas GF, et al. Late onset immune pancytopenia following bone marrow transplantation.Br J Haematol. 1991;78:268–274.
Ejstrud P, Kristensen B, Hansen JB, Madsen KM, Schonheyder HC, Sorensen HT. Risk and patterns of bacteraemia after splenectomy: a population-based study.Scand J Infect Dis. 2000;32:521–525.
McMullin M, Johnston G. Long term management of patients after splenectomy.BMJ. 1993;307:1372–1373.
British Committee for Standards in Haematology General Haematology Task Force. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy.Br J Haematol. 2003;120:574–596.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Zupan, I.P., Prentice, H.G., Pretnar, J. et al. Splenectomy for Severe Autoimmune Cytopenias after Allogeneic Stem Cell Transplantation: Case Report. Int J Hematol 82, 75–78 (2005). https://doi.org/10.1532/IJH97.E0430
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1532/IJH97.E0430