Successful allogeneic bone marrow transplantation for diamond-blackfan anemia complicated by severe cardiac dysfunction due to transfusion-induced hemochromatosis.
A 21-year-old man who was diagnosed with Diamond-Blackfan anemia at 2 years of age came to our hospital with the hope of undergoing bone marrow transplantation (BMT). He had been red cell transfusion-dependent for about 8 years; at presentation he had transfusion-induced hemochromatosis, a subsequent low left ventricular ejection fraction (LVEF) of 43%, and diabetes mellitus requiring insulin therapy. He received unrelated BMT with reduced intensity conditioning and sufficient GVHD prophylaxis. Regardless of the cardiac dysfunction, he had an uneventful course during pre- and post-grafting periods, and is currently doing well with an improved LVEF (55%), although he is still insulin dependent.
Tabata
Sumie
S
Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital.
Mori
Minako
M
Nagai
Yuya
Y
Hashimoto
Hisako
H
Arima
Hiroshi
H
Nagano
Seiji
S
Takiuchi
Yoko
Y
Inoue
Daichi
D
Kimura
Takaharu
T
Shimoji
Sonoko
S
Yanagita
Soshi
S
Ito
Kiminari
K
Matsushita
Akiko
A
Nagai
Kenichi
K
Takahashi
Takayuki
T
D029503:Anemia, Diamond-Blackfan / Q000628:therapy*; D016026:Bone Marrow Transplantation; D003920:Diabetes Mellitus / Q000188:drug therapy / Q000209:etiology; D006432:Hemochromatosis / Q000150:complications* / Q000209:etiology; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use; D007328:Insulin / Q000627:therapeutic use; D008297:Male; D065227:Transfusion Reaction; D014184:Transplantation, Homologous; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis / Q000209:etiology*; D055815:Young Adult
D007004:Hypoglycemic Agents; D007328:Insulin