Summary:
We report a 39-year-old female patient who underwent HLA-identical sibling allogeneic BMT for CML in accelerated phase. Severe pancytopenia refractory to G-CSF associated with progressive splenomegaly and RBC/platelet transfusion dependency were present from day +60 after BMT. MRD assessed by FISH and RT-PCR multiplex for BCR-ABL rearrangement was negative, and complete chimerism was documented by VNTR on days +100, +180, +360 and 2 years after BMT. Splenectomy was performed on day +225 and pancytopenia resolved but chronic extensive graft-versus-host disease developed, with hepatic cholestasis, diffuse scleroderma and sicca-like syndrome. She was sequentially and progressively treated with different immunosuppressive therapy combinations with no clear benefit. On day +940, she presented with infection over the previously present ulcers on both limbs, which culminated in septic shock and death on day +1041. We conclude that, although splenectomy may reverse poor graft function after allogeneic BMT, hyposplenism may trigger or worsen chronic extensive GVHD leading to increased morbidity and mortality.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$259.00 per year
only $21.58 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
Richard C, Romon I, Perez-Encinas M et al. Splenectomy for poor graft function after allogeneic BMT in patients with chronic myeloid leukemia. Leukemia 1996; 10: 1615–1618.
von Bueltzingsloewen A, Bordigoni P, Dorvaux Y et al. Splenectomy may reverse pancytopenia occurring after allogeneic BMT. Bone Marrow Transplant 1994; 14: 339–340.
Grigg AP, Berean K, Shore T, Phillips GL . Pancytopenia due to hypersplenism after allogeneic BMT. Bone Marrow Transplant 1992; 10: 177–179.
Cuthbert RJ, Iqbal A, Gates A et al. Functional hyposplenism following allogeneic BMT. J Clin Pathol 1995; 48: 257–259.
Ringden O, Nilsson B . Death by graft-versus-host disease associated with HLA mismatch, high recipient age, low marrow cell dose, and splenectomy. Transplantation 1985; 40: 39–44.
Bostrom L, Ringden O, Jacobsen N et al. A European multicenter study of chronic graft-versus-host disease. The role of cytomegalovirus serology in recipients and donors – acute graft-versus-host disease, and splenectomy. Transplantation 1990; 49: 1100–1105.
Michallet M, Corront B, Bosson JL et al. Role of splenectomy in incidence and severity of acute graft-versus-host disease: a multicenter study of 157 patients. Bone Marrow Transplant 1991; 8: 13–17.
Kalhs P, Schwarzinger I, Anderson G et al. A retrospective analysis of the long-term effect of splenectomy on late infections, graft-versus-host disease, relapse, and survival after allogeneic marrow transplantation for CML. Blood 1995; 86: 2028–2032.
Santos GW, Tutschka PJ, Brookmeyer R et al. Marrow transplantation for acute nonlymphocytic leukemia after treatment with busulfan and cyclophosphamide. N Eng J Med 1983; 309: 1347–1353.
Storb R, Deeg HJ, Whitehead J et al. Marrow transplantation for leukemia and aplastic anemia: two controlled trials of a combination of methotrexate and cyclosporine vs cyclosporine alone for prophylaxis of acute graft-vs-host disease. Transplant Proc 1987; 19: 2608.
Korngold R, Sprent J . Lethal GVHD across minor histo-compatibility barriers: nature of the effector cells and role of the H-2 complex. Immunol Rev 1983; 71: 5–29.
Rus V, Svetic A, Nguyen P et al. Kinetics of Th1 and Th2 cytokine production during the early course of acute and chronic murine graft-versus-host disease. J Immunol 1995; 155: 2396–2406.
Baumgartner JD, Glauser MP, Burgoblack AL et al. Severe cytomegalovirus infections in multiply transfused, splenectomized trauma patients. Lancet 1982; 2: 63–66.
Garcia-Girarlt E, Morales VH, Lasalvia E et al. Suppression of graft-versus-host reaction by a spleen extract. J Immunol 1972; 109: 878–880.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Rodrigues, C., Fermino, F., Vasconcelos, Y. et al. Refractory chronic GVHD emerging after splenectomy in a marrow transplant recipient with accelerated phase CML. Bone Marrow Transplant 32, 333–335 (2003). https://doi.org/10.1038/sj.bmt.1704122
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1038/sj.bmt.1704122