title: Immunophenotyping in myelodysplastic syndromes can add prognostic information to well-established and new clinical scores.
abstract: BACKGROUND
  myelodysplastic syndromes (MDS) are a heterogeneous group of hematopoietic clonal disorders. So, prognostic variables are important to separate patients with a similar biology and clinical outcome. We compared the importance of risk stratification in primary MDS of IPSS and WPSS with the just described revision of IPSS (IPSS-R), and examined if variables obtained by bone marrow immunophenotyping could add prognostic information to any of the scores.
  
  
  METHODS
  In this prospective study of 101 cases of primary MDS we compared the relation of patients' overall survival with WHO types, IPSS, IPSS-R, WPSS and phenotypic abnormalities of hematopoietic precursors. We examined aberrancies in myelomonocytic precursors and CD34(+) cells. Patients were censored when receiving chemotherapy or BM transplantation. Survival analysis was made by Cox regressions and stability of the models was examined by bootstrap resampling.
  
  
  RESULTS
  MEDIAN AGE: 64 years (15-93). WHO types: 2 cases of 5q- syndrome, 7 of RA, 64 of RCDM and 28 of RAEB. In the univariate Cox analysis, increasing risk category of all scores, degree of anemia, higher percentage of BM blasts, higher number of CD34(+) cells and their myeloid fractions besides increasing number of phenotypic abnormalities detected were significantly associated with a shorter survival. In the multivariate analysis comparing the three scores, IPSS-R was the only independent risk factor. Comparing WPSS with phenotypic variables (CD34(+)/CD13(+) cells, CD34(+)/CD13(-) cells and "total alterations") the score and "CD34(+)/CD13(+) cells" remained in the model. When IPSS was tested together with these phenotypic variables, only "CD34(+)/CD13(+) cells", and "total alterations" remained in the model. Testing IPSS-R with the phenotypic variables studied, only the score and "CD34(+)/CD13(+) cells" entered the model.
  
  
  CONCLUSIONS
  Immunophenotypic analysis of myelomonocytic progenitors provides additional prognostic information to all clinical scores studied. IPSS-R improved risk stratification in MDS compared to the former scores.
authors: Hematology and Hemotherapy Center, University of Campinas - Campinas, Sao Paulo, Brazil.
authors: Suiellen C
authors: SC
authors: Reis-Alves
authors: Fabíola
authors: F
authors: Traina
authors: Guilherme
authors: G
authors: Harada
authors: Paula M
authors: PM
authors: Campos
authors: Sara T O
authors: ST
authors: Saad
authors: Konradin
authors: K
authors: Metze
authors: Irene
authors: I
authors: Lorand-Metze
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018952:Antigens, CD34 / Q000276:immunology; D001853:Bone Marrow / Q000276:immunology* / Q000473:pathology; D016026:Bone Marrow Transplantation; D018826:CD13 Antigens / Q000276:immunology; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000276:immunology* / Q000401:mortality / Q000628:therapy; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012307:Risk Factors; D016019:Survival Analysis
chemical_list: D018952:Antigens, CD34; D018826:CD13 Antigens