| Myelodysplastic syndromes are a heterogenous group of haematologic malignancies characterized by a clonal abnormality of hematopoietic stem cells,that leads to bone marrow failure, peripheral cytopenia and may evolve into acute myeloid leukemia .According to FBA classification criteria, MDS was separated into 5 subsets :refractory anemia(RA), refractory anemia with ringed sideroblasts(RAS), refractory anemia with excess blasts(RAEB), refractory anemia with excess blasts in transforming(RAEB-t),chronic myelomonocytic leukemia (CMML).The onset of MDS before age 50 years is uncommon, MDS in children is less occur than in adults with a incidence rate of 2.7 per million.However,the incidence in children increases in recent years.There are many differences between childhood and adult MDS. Childhood MDS deteriorates more quickly and has much more possibility transforming into leukemia than adult MDS. Complete or partial deletion of chromosome 7 is the most prevalent karyotypic abnormality in MDS associated with diverse inherited diseases. Study suggested about 13.4 percents of children with MDS and 42.8%RAEB type patients possibly transformed into acute leukemia during 2~7 months. Because of without efficient therapy and poor global outcome of patients with MDS ,a major goal in this disease is to define efficient treatment directing against etiology and pathogenesis. The etiology of MDS is poorlyunderstood. Study suggested that chromosomal deletion and chromosomal loss, gene mutation, cells abnormal hyperplasia and apoptosis possibly take part in MDS etiology. Bone marrow microenvironment and immune factor implicated in bone marrow failure and many cytokine level is abnormal in MDS such as interleukin-l( IL-1), IL-2,IL-3,IL-6,IL-8, interferon-r (IFN-r),tumor necrosis factor-alfa(TNF- a )Jn acute leukemia TNF- a is involved in pathogenesis and disease progression. It is associated with the heavier tumor burden and may be a useful indication in determining the efficacy and prognosis. In recent years studies pay attention to the function of TNF- a in MDS pathogenesis, while there are no documents on it's relationship with disease progression and prognosis. Transforming growth factor- beta one(TGF- 3 i) is one of important factors associated with cell cycle. It has stimulative and inhibitive bifounction to cells.In acute leukemia patients its level is closely associated with the rate of commplete remission(CR).This study pay more attention to the relationship between TNF- a , TGF- 3 j and MDS in immunology and molecular biology point of view in order to provide theoretical base for differential diagnosis, patients condition evaluation, prognosis and new treatment.The perpheral blood serum levels and the bone marrow supernane levels of TNF- a and TGF- 31were measured by ELISA in 18 children MDS patients, meanwhile, the TNF- a and TGF- 3 i levels before and after treatment in 14 patients with MDS also detected. The relation of TNF- a and TGF- 3 i levels with MDS were analysized.The results showed:1. The serum levels of TNF- a in MDS children (184 ±21ng/L) was higher than the controls (74± 14ng/L), and had significant difference (PO.01) .In MDS children, the Serum TGF- 3 i level (13.46±3.33ug/L)was lower than the controls (44.26±4.43ug/L) ,and also had significant difference (PO.01) .2.Compared with those in normal controls (81 +20.5 ng/L), the bone marrow supernane levels of TNF-a (233 + 14 ng/L) were significant higher in MDS patients before treatment, and also higher than the serum levels (184 ± 21 ng/L) .At the same time ,it had positive correlation between serum levels ofTNF- a and bone marrow supernane levels of TNF- a (r=0.84, PO.01). The pretreatment levels of TGF- P i from bone marrow supernane (15.21 ±2.48 ug/L) was significantly lower than the normal controls (49.58 ±2.6 ug/L, PO.01) and higher than the serum levels ( 13.46 ±3.33ug/L) .There was also positive correlation between serum levels and bone marrow supernane levels(r=0.504, PO.05).3. The serum levels of TNF- a in 11 patients with RA and RAS (201 ± 11.4ng/L) was higher than that 7 patients with RAEB and RAEB-t (162±16.8 ng/L) ,but have no significant difference (P>0.05) .The serum levels of TGF- P i in RA and RAS patients was also higher than that in RAEB and RAEB-t patients and have no significant difference (P>0.05) .4.The TNF- a ,TGF- 3 i level alteration in MDS patients before and after treatment.The serum TNF-a level (80±11.2 ng/L) was significant decreased in steady group ,compared with the level in patients before treatment C190 ±18 ng/L), and have significant difference (PO.05) .However, the post treatment serum TNF- a was higher in active group (221 ±25ng/L) than pretreatment (162 ± 20.4ng/L) .In contrast to the pretreatment patients in remission group, there was a post-treatment increase in serum level of TGF- 3 i,but have no significant difference (P>0.05) .The serum level of TGF- 3 j in active patients was lower than the pretreatment level, also have no significant difference.In conclusion, our results have proved:1. TNF- a and TGF- 3 i take part in the pathogensis of MDS.2.There were difference of TNF- a and TGF- & \ levels in different types of MDS, but they can not be a parameter for typing.3. TNF- a can be used an indicator of evaluating curative effect and prognosis in MDS patients.There were some relations among TGF-3 ,level, MDS typing and patients' condition evaluation.
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