title: Refractory hyperhaemolysis in a patient with beta-thalassaemia major.
abstract: We report the case of a 1-year-old girl with newly diagnosed beta-thalassaemia major. Following an initial blood transfusion with phenotypically matched blood, she developed a haemolytic anaemia which progressed with subsequent transfusions. The Direct Antiglobulin test (DAT) was strongly positive with C3d and weakly with IgG. The only free antibodies detected were a weak anti-H and a weak cold auto-antibody, which did not exhibit a wide thermal range. The indirect Donath-Landsteiner and Ham's tests were negative. There was no sustained clinical response to steroids, immunoglobulin infusions or splenectomy. An HLA identical sibling donor was available for allogeneic bone marrow transplantation (BMT) and the haemolysis resolved during the immunosuppressive transplant conditioning. Such hyperhaemolysis without significant red cell alloantibodies has previously been reported in patients with sickle cell anaemia, but only rarely in patients with beta-thalassaemia major.
authors.affiliation: Manchester Children's Hospital, Pendlebury, Manchester, UK.
authors.forename: J D
authors.initials: JD
authors.lastname: Grainger
authors.forename: Y
authors.initials: Y
authors.lastname: Makar
authors.forename: A
authors.initials: A
authors.lastname: McManus
authors.forename: R
authors.initials: R
authors.lastname: Wynn
mesh_terms: D001323:Autoantibodies / Q000097:blood; D001803:Blood Transfusion; D016026:Bone Marrow Transplantation / Q000276:immunology / Q000502:physiology*; D015933:Complement C3d / Q000032:analysis; D005260:Female; D006461:Hemolysis; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D019520:Living Donors; D009679:Nuclear Family; D013156:Splenectomy; D017086:beta-Thalassemia / Q000097:blood* / Q000276:immunology / Q000628:therapy*
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D015933:Complement C3d