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.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Toren A,Or R,Kapelushnik J,Chividalli G,Aku M,Slavin S,Nagler Adoi
10.1002/(SICI)1096-8652(199602)51:2<166::AID-AJH13subject
Has Abstractpub_date
1996-02-01 00:00:00pages
166-7issue
2eissn
0361-8609issn
1096-8652pii
10.1002/(SICI)1096-8652(199602)51:2<166::AID-AJH13journal_volume
51pub_type
杂志文章abstract::There is a high prevalence of thalassemia in the Taiwan area. Prenatal diagnosis of severe forms of thalassemia is important for the prevention of this disease. We performed prenatal diagnosis in 167 cases, of which 59 cases were diagnosed by chorionic villi biopsy, 91 cases by amniotic fluid analysis, and 17 cases by...
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