Refractory chronic GVHD emerging after splenectomy in a marrow transplant recipient with accelerated phase CML
Data
2003-08-01
Autores
Fermino, Fabiana Aidar 
Vasconcelos, Yuri
Tipo
Artigo
Título da Revista
ISSN da Revista
Título de Volume
Resumo
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.
Descrição
Citação
Bone Marrow Transplantation. London: Nature Publishing Group, v. 32, n. 3, p. 333-335, 2003.