title: Late onset transfusion-dependent anaemia with thrombocytopenia secondary to marrow fibrosis and hypoplasia associated with chronic graft-versus-host disease.
abstract: Late onset transfusion-dependent anaemia is very rare in the presence of sustained engraftment after HLA-identical sibling bone marrow transplantation. The only previously described consistent cause is pyridoxine-responsive sideroblastic anaemia. We describe here a second cause, marrow hypoplasia and fibrosis, occurring in association with extensive chronic graft-versus-host disease (GVHD). A 20-year-old Caucasian male who received cyclophosphamide and fractionated total body irradiation followed by an unmanipulated HLA-identical sibling marrow transplant from his sister for acute nonlymphoblastic leukaemia in first remission developed chronic GVHD of the skin and mouth at day 101 post-transplant. At day 689 post-transplant, he developed leuco-erythroblastic anaemia with thrombocytopenia, due to patchy marrow hypoplasia and fibrosis. Between days 689 and 1987 post-transplant he received 71 units of packed red cells, requiring transfusion approximately monthly. He remains well although still on prednisone for chronic GVHD of skin and is receiving desferrioxamine 4 g five nights/week i.v. as prophylaxis for iron overload.
authors.affiliation: Department of Haematology, St Vincent's Hospital, Sydney, Australia.
authors.forename: K
authors.initials: K
authors.lastname: Atkinson
authors.forename: A
authors.initials: A
authors.lastname: Dodds
authors.forename: A
authors.initials: A
authors.lastname: Concannon
authors.forename: J
authors.initials: J
authors.lastname: Biggs
mesh_terms: D000328:Adult; D000740:Anemia / Q000209:etiology*; D001803:Blood Transfusion; D001853:Bone Marrow / Q000473:pathology*; D016026:Bone Marrow Transplantation; D002908:Chronic Disease; D005355:Fibrosis; D006086:Graft vs Host Disease / Q000150:complications*; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000628:therapy; D008297:Male; D013921:Thrombocytopenia / Q000209:etiology*