title: Normalization of serum lactic dehydrogenase in beta-thalassemia patients following bone marrow transplantation.
abstract: Serum lactic dehydrogenase (LDH) levels are mildly elevated in beta-thalassemia major due to ineffective erythropoiesis. We reviewed the charts of 15 consecutive thalassemic children who underwent allogeneic, T-cell-depleted bone marrow transplantation (BMT) in our department during the last 3 years. Eleven patients had successful engraftment and are alive and well without evidence of disease, according to physical examinations, blood counts, and polymerase chain reaction (PCR) tests, with a median follow-up of 2 years. Two patients died due to transplantation-related complications, and two rejected the graft and received their backup autologous marrow. The LDH levels in the transplanted patients gradually decreased from an average of 952 +/- 155 IU/L 10 days pre-transplant (N = 300-620) to 426 +/- 56 IU/L at the day of transplantation, and stayed at approximately the same level post-transplant (489 +/- 55 IU/L). By contrast, the LDH levels reverted to the pre-transplant value in those patients who rejected their marrow. The significance of this clinical observation for the pathophysiologic mechanism of intramedullary hemolysis and ineffective erythropoiesis in beta-thalassemia major is discussed.
authors.affiliation: Department of Bone Marrow Transplantation, Hadassah University Hospital, Jerusalem, Israel.
authors.forename: A
authors.initials: A
authors.lastname: Toren
authors.forename: R
authors.initials: R
authors.lastname: Or
authors.forename: J
authors.initials: J
authors.lastname: Kapelushnik
authors.forename: G
authors.initials: G
authors.lastname: Chividalli
authors.forename: M
authors.initials: M
authors.lastname: Aku
authors.forename: S
authors.initials: S
authors.lastname: Slavin
authors.forename: A
authors.initials: A
authors.lastname: Nagler
mesh_terms: D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood*; D008297:Male; D017086:beta-Thalassemia / Q000201:enzymology / Q000628:therapy*
chemical_list: D007770:L-Lactate Dehydrogenase