Allogeneic peripheral stem cell transplantation in a case of hereditary sideroblastic anaemia.
We report on a case of pyridoxine refractory hereditary sideroblastic anaemia (HSA) in a 19-year-old man who underwent peripheral blood stem cell transplantation (PBSCT) from his HLA-identical brother. By using short tandem repeat polymorphism, 100% donor cells were observed in peripheral blood on day +21; bone marrow showed mixed chimaerism from day +21 to day +221, when 100% cells of donor origin were observed. The patient developed extensive chronic graft-versus-host disease with favourable response to treatment. When the haemoglobin range was normal, a programme of phlebotomies reduced serum ferritin levels. Three years after transplantation, the patient has an ECOG rating of 0, with completely normal haemoglobin values (15 g/dl). To our knowledge, this is the first PBSCT reported in a case of hereditary sideroblastic anaemia.
González
M I
MI
Servicio de Hematología, Hospital Clínico Universitario, Salamanca, Spain.
Caballero
D
D
Vázquez
L
L
Cañizo
C
C
Hernández
R
R
López
C
C
Izarra
A
A
Arroyo
J L
JL
González
M
M
García
R
R
San Miguel
J F
JF
D000328:Adult; D000756:Anemia, Sideroblastic / Q000235:genetics* / Q000276:immunology / Q000628:therapy*; D001803:Blood Transfusion; D016572:Cyclosporine / Q000627:therapeutic use; D005500:Follow-Up Studies; D006086:Graft vs Host Disease / Q000276:immunology; D006680:HLA Antigens / Q000276:immunology; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D013256:Steroids / Q000627:therapeutic use; D014184:Transplantation, Homologous
D006680:HLA Antigens; D007166:Immunosuppressive Agents; D013256:Steroids; D016572:Cyclosporine