TREATMENT OF SEVERE AUTOIMMUNE CYTOPENIAS AFTER ALLOGENEIC STEM CELL TRANSPLANTATION – REPORT OF TWO CASES
Abstract
Background. Autoimmune cytopenias, thrombocytopenia, anemia and neutropenia, are rare but serious complications after stem cell transplantation (SCT). There are only a few reports concerning their treatment. We performed splenectomy in two patients with severe autoimmune cytopenias after allogeneic SCT resistant to immunosuppressive treatment.
Patients, methods and results. First patient had unrelated alloSCT at Royal Free Hospital London for chronic granulocytic leukemia (CGL) in July 2000. Post-transplant period was complicated with cytomegalovirus reactivation and septicemia. Seven months later RBC and platelet counts went down. Direct Coomb’s test was intermittently positive. She was resistant to steroids and high dose immunoglobulin. Splenectomy was performed in February 2001. After splenectomy hemoglobin concentration and platelet count improved. Her blood counts remained stable with hemoglobin about 110 g/L and platelets over 100 x109/L. She continued therapy with Itraconazol, Valacyclovir and Penicillin. Three months later she was readmitted for E. Coli fulminated septic infection with fatal outcome.
Second patient had related alloSCT at University Medical Centre Ljubljana for CGL in January 2003. Post-transplant course was uneventful. Seven months later he was readmitted for retinal bleeding with severe thrombocytopenia with positive anti-platelet antibodies. He was resistant to steroids and high dose immunoglobulin. Splenectomy was performed in September 2003. His platelet count normalized and remains stable so far. He continues therapy with Itraconazol, Valacyclovir and Penicillin and didn’t experience any serious infection.
Conclusions. We assume that splenectomy is an efficient treatment for resistant immune cytopenias after alloSCT. However, severe late infections may compromise the outcome.
Downloads
References
Deeg JH. Delayed complications after hematopoietic cell transplantation. In: Thomas ED, Blume KG, Forman SJ. Hematopoietic cell transplantation. Oxford: Blackwell Science, 1999: 776–88.
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: 469–75.
Atkinson K. Reconstruction of the haemopoietic and immune systems after marrow transplantation. Bone Marrow Transplant 5: 209–26.
Sherer Y, Shoenfeld Y. Autoimmune diseases and autoimmunity post bonemarrow transplantation. Bone Marrow Transplant 1998; 22:873–81.
Itoh M, Takahashi T, Sakaguchi N et al. Thymus and autoimmunity: production of CD4+CD25+ naturally anergic and suppressive T cells as a key function of the thymus in maintaining immunologic self-tolerance. J Immunol 1999; 162: 5317–26.
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: 745–7.
Ishikawa F, Shigematsu H, Gondo H et al. Autoreactive antibodies following autologous peripheral blood stem cell transplantation. Bone Marrow Transplant 1998; 22: 729–31.
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: 739–41.
Ting SS, Ziegler JB, Vowels MR. Acquired autoimmune thrombocytopenia post-bone marrow transplantation for severe combined immunodeficiency. Bone Marrow Transplant 1998; 21: 841–3.
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: 173–4.
Klumpp TR, Herman JH, Macdonald JS, Schnell MKMullaney M, Man KF. Autoimmune neutropenia following peripheral blood stem cell transplantation. Am J Hematol 1992; 41: 215–7.
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: 297–9.
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: 1309–15.
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: 531–6.
Drobyski WR, Potluri J, Sauer D, Gottschall JL. Autoimmune hemolytic anemia following T-cell-depleted allogeneic bone marrow transplantation. Bone Marrow Transplant 1996; 17: 1093–9.
Jillella AP, Kallab AM, Kutlar A. Autoimmune thrombocytopenia following autologous hematopoietic cell transplantation: review of literature and treatment options. Bone Marrow Transplant 2000; 26: 925–7.
Tosi P, Bandini G, Tazzari P et al. Autoimmune neutropenia after unrelated bone marrow transplantation. Bone Marrow Transplant 1994; 14: 1003–4.
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: 237–9.
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: 337–40.
Damoiseaux JG, van Breda Vriesman PJ. Pathogenesis of cyclosporin Ainduced 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: 268–74.
Ejstrud P, Kristensen B, Hansen JB, Madsen KM, Schonheyder HC, Sorensen HT. Risk and patterns of bacteraemia after splenectomy: a populationbased study. Scand J Infect Dis. 2000; 32: 521–5.
McMullin M, Johnstone G. Long term management of patients after splenectomy. British Medical Journal 1993; 307: 1372–3.
British Society of Haematology. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. British Journal of Haematology 2003; 120: 574–96.
The Author transfers to the Publisher (Zdravniški vestnik/Slovenian Medical Journal) all economic copyrights following form Article 22 of the Slovene Copyright and Related Rights Act (ZASP), including the right of reproduction, the right of distribution, the rental right, the right of public performance, the right of public transmission, the right of public communication by means of phonograms and videograms, the right of public presentation, the right of broadcasting, the right of rebroadcasting, the right of secondary broadcasting, the right of communication to the public, the right of transformation, the right of audiovisual adaptation and all other rights of the author according to ZASP.
The aforementioned rights are transferred non-exclusively, for an unlimited number of editions, for the term of the statutory
The Author can make use of his work himself or transfer subjective rights to others only after 3 months from date of first publishing in the journal Zdravniški vestnik/Slovenian Medical Journal.
The Publisher (Zdravniški vestnik/Slovenian Medical Journal) has the right to transfer the rights, acquired parties without explicit consent of the Author.
The Author consents that the Article be published under the Creative Commons BY-NC 4.0 (attribution-non-commercial) or comparable licence.
