The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal myeloid

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The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal myeloid haemopathies characterized by defective differentiation of hematopoietic cells and expansion of the abnormal clone. (14). An Italian group evaluated the prognostic value of the new WHO classification and showed that WHO morphologic subgroups, IPSS cytogenetic categories, and red blood cell (RBC) transfusion-dependency each had prognostic value in MDS (15). They also showed that RBC transfusion-dependence with development of secondary iron overload had a worse prognosis in multivariate analysis. These workers extended this analysis to evaluate significant prognostic factors taking into account changes over time to develop the WPSS, a dynamic MDS prognostic model that stratified patients into five risk groups (2). In multivariate analysis, the most important variables were WHO subgroups, IPSS karyotype, and transfusion requirement. Age had an effect on survival in the lower risk groups. Subsequently the latter parameter was replaced by haemoglobin (Hgb) level, changing the transfusion-dependency variable to Hgb <9 g/dL for males and <8 g/dL for females (16). The WPSS was also validated and pre-transplant score shown to have prognostic value in the post-transplantation outcomes of patients with MDS (17). The main advantage of the WPSS was the ability to be used for serial prognostication. It had similar limitations as the IPSS and did not account for degree of cytopenias, excluded RAEB-t patients, and relied on detailed morphologic analysis (e.g., Ranirestat IC50 dysplasia) to determine WHO subtype that has not been universally discernable. M.D. Anderson Lower-Risk MDS Prognostic Scoring System (MDA-LR) In an effort to further characterize the prognosis of lower-risk IPSS patients, patients with IPSS low or INT-1 risk disease referred to the M.D. Anderson Cancer Center Ranirestat IC50 (MDACC) were evaluated for characteristics that predicted shorter survival (3). Multivariate analysis of clinical characteristics exhibited that unfavourable cytogenetics, Hgb, platelet (Plt) count, and BM blasts percentage were significantly associated with survival. These variables were weighted and used to develop the MDA-LR which stratified patients into three risk groups. This analysis also included patients with CMML and secondary MDS (sMDS). Limitations were similar to the IPSS and WPSS. Global M.D. Anderson Risk Model Score for MDS (MDAS) The group at MDACC also evaluated prognostic features in MDS patients referred at any time in their disease course in an attempt to improve upon the IPSS (4). Multivariate analysis identified several impartial adverse factors as continuous and categorical values including Eastern Cooperative Oncology Group (ECOG) performance status, older age, thrombocytopenia, anaemia, increased BM blasts, leucocytosis, chromosome 7 or complex abnormalities, and prior transfusions. These disease and host factors were weighted and used to develop the MDAS model which stratified patients into four risk categories. The models prognostic significance did not change when the WHO-defined AML patients were excluded, and importantly, the model was able to stratify CMML and sMDS patients into the four risk categories similar to the general populace studied. This model was validated at another institution (18). The MDAS was more complex compared to the simpler IPSS score and the impact of prior therapy around the analysis of prognosis in this patient populace was unclear. The overall advantages of this model were the inclusion of patient populations not included in the prior IPSS or WPSS models, ability of this model to be used for dynamic prognostication, and identification of lower-risk IPSS patients with higher risk features. French Prognostic Scoring System (FPSS) The Groupe Francophone des Myelodysplasies (GFM) evaluated IPSS INT-2 or high risk patients treated with 5-azacitidine (AZA) for prognostic factors affecting response and survival (5). Multivariate analysis showed that ECOG performance status, IPSS cytogenetic risk, presence of circulating blasts, and RBC transfusion dependency were prognostic for survival. In addition, achievement of any type of haematological improvement (HI) in patients without complete or partial remission was associated with improved OS. Combining and weighting those factors affecting OS led to development of the FPSS, which discriminates three prognostic groups. This score was validated Ranirestat IC50 in an independent set of patients from the AZA-001 trial and retained its prognostic significance in longer follow-up of the same patient cohort (19, 20). However in a recent ECOG study, analysis indicated no improvement in OS prediction Rabbit Polyclonal to MINPP1 for the FPSS over the IPSS-R in AZA-treated patients (21). Chronic Myelomonocytic Leukaemia (CMML) Prognostic Scoring System (CPSS) Like MDS, CMML is a heterogeneous group of diseases with significant clinical variability, survival, and risk of transformation to AML. Previous MDS prognostic models differ with respect to CMML. The IPSS excluded MPN-like CMML (WBC >13.