HEMATOLOGIC RECONSTITUTION IN THALASSEMIA MAJOR PATIENTS WHO UNDERWENT BONE-MARROW TRANSPLANTATION

Citation
D. Demattia et al., HEMATOLOGIC RECONSTITUTION IN THALASSEMIA MAJOR PATIENTS WHO UNDERWENT BONE-MARROW TRANSPLANTATION, International journal of pediatric hematology/oncology, 3(4), 1996, pp. 225-228
Citations number
13
Categorie Soggetti
Oncology,Pediatrics,Hematology
ISSN journal
10702903
Volume
3
Issue
4
Year of publication
1996
Pages
225 - 228
Database
ISI
SICI code
1070-2903(1996)3:4<225:HRITMP>2.0.ZU;2-A
Abstract
We evaluated the hematologic reconstitution in a group of subjects wit h thalassemia major who underwent bone marrow transplantation. To achi eve this goal we studied 8 boys and 10 girls born during 1966 through 1990, 8 of whom received transplants from heterozygous for beta-thalas semia donors and 6 of whom received transplant from normal donors. We evaluated the following hematologic parameters in all patients: hemogl obin, red blood cell count, red blood cell mean corpuscular volume (MC V), red blood cell mean corpuscular hemoglobin, hemoglobin F, and hemo globin A2. Both groups of patients, those receiving transplants from n ormal donors and those receiving transplants from heterozygous for bet a-thalassemia donors, showed a statistically significant increase in r ed blood cell count, reaching values that overlapped those of the resp ective donors at the 24th month. Both groups of patients also showed a statistically significant increase in hemoglobin values, reaching val ues that overlapped those of the donors, respectively, at the 6th and 24th months. We noted also an initial absolute macrocytosis (MCV: 97.5 0 fl) in patients who received transplants from normal donors and a re lative macrocytosis in those who received transplants from heterozygou s for beta-thalassemia donors (MCV: 71.17 fl). A progressive decrease appeared successively in patients receiving transplants from normal do nors in the first 24 months. In those receiving transplants from heter ozygous for beta-thalassemia donors this decrease is equally visible i n the first 24 months after bone marrow transplantation. In both group s of patients, values overlapped those of the donors at the 24th month . Both groups of patients also showed a statistically significant decr ease in mean corpuscular hemoglobin values. No significant differences in hemoglobin F and A2 values at 24 months after bone marrow transpla ntation were noted in both groups, compared with their donors. The hem atologic reconstitution in thalassemia major patients is more rapid in those receiving transplants from normal donors rather than those rece iving transplants from heterozygous for beta-thalassemia donors, and t he hematologic values remained similar to those of the respective dono rs.