Clinical Investigation Of Myelodysplastic Syndrome With Autoimmune Disease

Myelodysplastic syndromes (MDS) are clonal disorders of haematopoietic stem cells characterized by ineffective and dysplastic haematopoiesis and peripheral cytopenias , 20%~40% of which transform into acute leucemi(aAL).The association of myelodysplasia and immunological abnormality disorders has been reviewed by Hamblin who first described two cases of autoimmune haemolytic anaemia in 104 patients with MDS. On the other hand, asymptomatic immunological abnormalities, first recognized by Mufti et al.Since then this association has been further highlighted in the medical literature by sporadic case reports and several retrospective studies. The incidence of MDS with AIDs in previous studies has been estimated more than 10%,and the underlying pathogenesis and prognostic significance of which still remain controversial,and it is known to be associated with significant abnormalities related to the immune system. In previous studies, the RA subcategory displayed the greatest number of AIDs cases,and sometimes the RAEB subcategory displayed the greatest number of AIDs cases.Clinical manifestations of such phenomena may include rheumatoid arthritis, Raynaud phenomenon,skin vasculitis, peripheral polyneuropathy, inflammatory bowel disease and even classical connective tissue disorders such as relapsing polychondritis.Asymptomatic immunological abnormalities include antinuclear antibody ,hype/hypoglobulinemia,positive rheumatoid factor or hemolysis test.Some scholars proposed that,though the patients with AIDs were not statistically different in Leukaemic transformation,life span decurtates obviously.while Giannouli etal proposed that patients with AIDs were not statistically different in survival from patients without AIDs,the RAEB subcategory displayed the greatest number of AIDs cases, but the largest number of patients in this study was in this subcategory. MDS patients in clinical researchs publicated at present, which were classified according to the French–American–British (FAB)classification,and there are not according to WHO(2000) classification in MDS patienrs with AIDs.Objective:In this retrospective study we tried to approach incidence of MDS with AIDs and relationship between the two diseases.Patients-Methods:Between January ,1999 and June ,2007, a total of 298 with MDS were subjected to a retrospective study,which were classified into three groups A(MDS with AIDs),B(MDS with asymptomatic immunological abnormalities ) and C(MDS without autoimmunity.We used the WHO(2000) classification which divide MDS into RA,RCMD, RARS, RCMD-RS, RAEB-1, RAEB-2, MDS-U and 5q-syndrome. we analyse the clinical characteristics of MDS patients with AID or asymptomatic immunological abnormalities, that include general document, clinical manifestation, blood routine examination , peripheral blood cells morphology examination , BM cytology examination , BM patho-examination , cytogenetics examination ,turnover and prognosis . and Immoral immunity disorders were systematically searched during MDS and conversely MDS searched during cytopenia . In the aspect of statistics, the software of statistics SPSS13.0 was used,the data is presented in mean±standard deviation , while Chi-square test was employed to test the significance of the numeration data .P<0.05 was considered to be statistical significance in all of the results.Results: The incidence of MDS patients with AID is 3.69% in our hospital,which is lower than fomer recorder.The incidence of MDS patients with asymptomatic immunological abnormalities is 25.53%. female patients who have these clinical manifestation are more than male.Autoimmune hemolytic anemia, Raynaud's phenomenon,Graves diseases is more than other autoimmune disease in our cases . Asymptomatic immunological abnormalities include antinuclear antibody,hype/hypoglobulinemia positive rheumatoid factor,hemolysis test or platelet antibody. the largest number of patients in this study was in subcategory of RCMD. On comparing features between the groups, we were unable to identify any particular difference (P>0.05)concerning age,peripheral blood cells morphology examination,BM cytology examination,BM patho-examination, cytogenetics examination , Leukaemic transformation , and prognosis, exclude sex.Conclusion:①The incidence of MDS patients with AID is 3.69%. The incidence of MDS patients with asymptomatic immunological abnormalities is 25.53%.female patients who have these clinical manifestation are more than male。②Autoimmune hemolytic anemia, Raynaud's phenomenon,Graves diseases is more than other autoimmune disease in our cases.③The RCMD subcategory displayed the greatest number of AIDs cases.④There was no significant difference in hemogram and bone marrow hyperplasity.but the incidence rate of chromosomal abnormality is higher.⑤Glucocorticosteroid is more effective in tpatients of asymptomatic immunological abnormalities.⑥There was no significant difference Leukaemic transformation and survival.