Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The HLA-A and -B antigens of 99 Greek patients with transfusion dependent homozygous beta thalassaemia were determined. The HLA antigen distribution in thalassaemic patients with a severe transfusion siderosis and in patients without signs of siderosis were compared to that of 400 healthy unrelated controls from the same population. There is an increase of HLA-B 14 antigen in both groups of thalassaemics as compared with the controls. No significant difference exists in the distribution of all the other HLA antigens between the two sub-groups of thalassaemics or with the controls.
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PMID:Iron stores in multi-transfused thalassaemic patients seem not to be influenced by the HLA system. 1120 31

Six transfusion-dependent beta-thalassemia major patients were treated with allogeneic peripheral blood stem cell (PBSC) transplant. The donors were HLA identical siblings except one donor who was a father with one-antigen mismatch of HLA-B loci. The donors were mobilized with G-CSF and PBSC was infused without manipulation. Engraftment was documented in all patients. Acute graft versus host disease (GvHD) was present in 4 patients but could be controlled with steroid or/and ATG. One patient died of hepatic veno-occlusive disease (HVOD) and survivors were all transfusion independent (ex-thalassemia). Chronic GvHD occurred in one patient. Allogeneic PBSC transplantation could achieve disease-free survival in beta-thalassemia major patients.
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PMID:Allogeneic peripheral blood stem cell transplantation in beta-thalassemia. 1221 90

In Vietnam, the first three cases of Allo-BMT were successfully performed in 1995 at the Blood Transfusion and Hematology Hospital (BT-H) of Ho Chi Minh City. Donors were HLA fully matched siblings (HLA-A, HLA-B and HLA-DRB1). The patients were a 26-year-old man with CML in chronic phase (CP), a 12-year-old woman with beta-thalassemia/Hb E and a 9-year-old girl with beta-thalassemia/Hb E. All patients were engrafted with the median time to recover ANC>0.5 x 10(9)/l, and platelet count >20 x 10(9)/l was 16 and 38 days. At 12 years after transplantation, all three patients are alive and well. Today, Vietnam has five SCT centers; in the north, there are three centers: 108 Military Hospital, Pediatric Institute and Blood transfusion and Hematology Institute; in the middle of Vietnam is Hue Hospital and in the south, the BT-H Hospital of Ho Chi Minh City. Until now, 65 patients have had SCT in Vietnam; among them, 52 patients had SCT at the BT-H Hospital, Ho Chi Minh City. Because of no connection of data between different SCT centers, we present here only the results performed at the BT-H Hospital, Ho Chi Minh City. With Allo-SCT we performed 19 cases with 3 procedures: BMT (4 cases), PBSC (6 cases) and cord blood transplantation (9 cases); patients were diagnosed with AML (n=7), ALL (n=1), CML (n=5) and beta-thalassemia (n=6). Following transplantation, 7 patients (36.84%) relapsed, 12 (63.16%) remained alive and overall survival times: 6.81+/-1.35 years, disease-free survival times: 6.69+/-1.4 years (range 0.5-12 years). With Auto-SCT: since November 1996, we have performed 33 cases of autologous PBSC transplantation consisting of without cryopreservation (24 cases) and with cryopreservation (9 cases); patients were diagnosed with AML in CR1 (n=21), ALL in CR1 (n=6), CML in CP (n=5) and non-Hodgkin's lymphoma in CR1 (n=1). The median age of the patients was 35 years (range 18-46). The median time to recover ANC >0.5 x 10(9)/l and platelet count >20 x 10(9)/l was 14 days (range 9-25 days) and 35 days (range 9-120 days). Following transplantation, 18 patients (54.50%) relapsed, 15 (45.45%) remained alive and overall survival times: 5.74+/-0.82 years and disease-free survival times: 5.48+/-0.92 years. There was no statistically significant difference of overall survival and disease-free survival between Allo-SCT and Auto-SCT procedures (P>0.05). These preliminary data suggest that HSCTs have been used as one of the standard treatments for hematological diseases and malignancies in Vietnam and that cord blood is an alternative source of hematopoietic stem cells for allogeneic transplantation in children.
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PMID:Current status of hematopoietic stem cell transplantations in Vietnam. 1872 91