title: Refractory chronic GVHD emerging after splenectomy in a marrow transplant recipient with accelerated phase CML.
abstract: 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.
authors.affiliation: Hospital São Paulo, São Paulo, Brazil.
authors.forename: C A
authors.initials: CA
authors.lastname: Rodrigues
authors.forename: F A
authors.initials: FA
authors.lastname: Fermino
authors.forename: Y
authors.initials: Y
authors.lastname: Vasconcelos
authors.forename: J S R
authors.initials: JS
authors.lastname: de Oliveira
mesh_terms: D000328:Adult; D001752:Blast Crisis; D016026:Bone Marrow Transplantation; D002908:Chronic Disease; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology*; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000150:complications / Q000473:pathology / Q000628:therapy*; D013156:Splenectomy / Q000009:adverse effects*; D014184:Transplantation, Homologous