Improving strategies for the prognostic evaluation of myelodysplastic syndromes patients

Resumen

Myelodysplastic syndromes (MDS) comprise a heterogeneous group of myeloid disorders with heterogeneous clinical manifestations and outcomes, ranging from asymptomatic patients with long-life expectancy to those with profound cytopenias and high risk to evolve into acute myeloid leukemia. Classically, treatment options have been based on risk prognostic stratification according to the International Prognostic Scoring System (IPSS), being conservative in lower-risk [IPSS low and intermediate-1 (IPSS <1.5 points)], whereas for higher-risk patients [IPSS intermediate-2 and high (IPSS >1.5 points)] an intensive approach was proposed. Nevertheless, the IPSS has been recently revised (IPSS-R) in which lower-risk MDS are those assigned to the IPSS-R very low and low categories (IPSS-R <3.5), whereas higher-risk MDS those assigned to the IPSS-R high and very high categories (IPSS-R >4.5). Importantly, the newer IPSS-R includes a new risk category, the intermediate group (IPSS-R 3.5–4.5), in which outcomes and clinical interventions are not completely defined. Therefore, and from the clinical standpoint, a proper IPSS-R threshold that dichotomized lower-risk MDS vs higher-risk MDS is desirable. On the other hand, other concomitant conditions to the MDS can have prognostic implications. Among them, autoimmune disorders (AD) have been described in MDS patients, although their real incidence and prognostic impact in this setting are not completely understood. Against this background, we have revisited the prognostic factors related to the MDS. In one hand, the prevalence, clinical characteristics and outcomes of AD in MDS patients. In addition, which is the best IPSS-R threshold that dichotomizes MDS in low vs high-risk patients. According to our results, AD are frequent in MDS conferring an adverse prognostic impact, whereas an IPSS-R of 3 points is the threshold that best divide patients into low and high-risk subgroups. Altogether, these results could change the clinical practice of patients with MDS, since those patients with AD or those with an IPSS-R >3 should be considered as high-risk patients and could be candidates for an intensive approach.