Myelodysplastic syndromes: classification and prognostic scoring systems and their applicability in Indian scenario-experience from a tertiary care center

摘要

The myelodysplastic syndromes (MDS) are a group of clonal disorders characterized by ineffective haematopoiesis, cytopenias, morphologic dysplasia and leukemic transformation. Difficulties exist in classifying and prognosticating MDS. This study was done to evaluate FAB and WHO classifications and the role of infection especially tuberculosis contributing to secondary myelodysplasia. The clinico-hematological profile of all cases (n=78) of MDS diagnosed over the last one and a half years was analyzed. This included 73 cases of primary MDS and five cases of infection associated myelodysplasia. There were 50 male and 28 female patients. Mean age at presentation was 46·1 years (range: 9 to 82 years). Out of 73 cases, two progressed to AML during the study period. Seventy cases could be classified based on FAB and 62 based on WHO criteria. Five cases of FAB-RAEBt were AML by FAB. One case not classifiable as per FAB could be categorized by WHO and four cases not classifiable as WHO could be categorized by FAB classification. All fulfilled the minimal diagnostic criteria for MDS. The commonest subtype of MDS was RA by FAB (55·7%) and RCMD (21%) and MDS-U (21%) by WHO. Four patients with tuberculosis and one with HIV showed significant myelodysplasia along with reactive changes. The consensus proposal of minimal diagnostic criteria for MDS was most helpful in cases difficult to diagnose and classify. Coexisting infection especially tuberculosis causing secondary myelodysplasia needs to be kept in mind especially in the Indian subcontinent.