Abstract
Backgroundmyelodysplastic 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.MethodsIn 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.Resultsmedian 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.ConclusionsImmunophenotypic 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.
Highlights
Myelodysplastic Syndromes (MDS) constitute a wide spectrum of hematopoietic clonal disorders with a variable clinical course [1,2,3,4,5]
The severity of peripheral blood counts (PB) cytopenias, the percentage of bone marrow (BM) blasts and the kind of cytogenetic abnormalities found have long been recognized as independent prognostic factors in MDS, and have been included in the currently used prognostic scores, such as IPSS and WHO classification-based Prognostic Scoring System (WPSS) [4,5,6,7,8,9,10]
The IPSS, [6] that is currently the most frequently used risk stratification score for MDS, is based on the number of cytopenias found in peripheral blood counts (PB), percentage of bone marrow (BM) blasts and kind of cytogenetic abnormalities
Summary
Myelodysplastic Syndromes (MDS) constitute a wide spectrum of hematopoietic clonal disorders with a variable clinical course [1,2,3,4,5]. The IPSS, [6] that is currently the most frequently used risk stratification score for MDS, is based on the number of cytopenias found in peripheral blood counts (PB), percentage of bone marrow (BM) blasts and kind of cytogenetic abnormalities. This score has recently been revised (IPSS-R) [9] as the importance of karyotype abnormalities was underscored in the former classification system. A considerable proportion of cases with IPSS intermediate risk switched to a higher risk category
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