Consideration of iron-chelation (IC) in transfusion-dependent patients is recommended in most clinicalpractice guidelines on myelodysplastic syndromes (MDS). The financial impact of IC on health-care systems is predicted through economic modeling, but an analysis based on actual prevalence is lacking. Here, we have investigated the potential drug-costs and need for IC in a cohort of 189 United Kingdom-based MDS patients diagnosed from 2000 to 2010. Patients with low or intermediate-1 IPSS scores were identified as eligible for IC if 24 red cell units (RCU) had been transfused over 12 consecutive months or the transfusion-intensity averaged 2 RCU per month. Drug-costs were calculated from the time patients qualified for IC until death or last follow-up. In 159 patients with low/intermediate-1 MDS, survival was superior with a low IPSS score (P 5 0.014), age <70 years (P 5 0.043), transfusion-independence at diagnosis (P 5 0.0056) and transfusion-intensity of <2 RCU per month (P 5 0.009). Reflecting the time elapsed since diagnosis, longer survival was observed with a cumulative red cell load of 75 U (P 5 0.046). By logistic-regression analysis, transfusion-intensity independently predicted survival (P 5 0.0035) in low and intermediate-1 risk MDS patients. Forty-one patients fulfilled criteria for consideration of IC. Of these, 6 patients died within 1 month; 35 patients survived for a median of 16 months (range 1-61). Had patients commenced IC, the anticipated drug-costs alone would have been $526,880-$2,064,800 over 10 years. The lack of association between cumulative transfusion-load and survival calls for a prospective evaluation of the cost-utility of IC in patients surviving long-term, to enable evidencebased recommendations in MDS management. Am. J. Hematol. 86:406-410, 2011. V
The differential diagnoses and histopathological findings of a case of recurrent parotid swelling, syncope, arthritis and nephrotic syndrome will be discussed. Primary Sjögren's syndrome is rarely associated with granulomatous inflammation, panniculitis and systemic amyloidosis. Multi‐organ failure, in particular cardiac amyloidosis, is potentially fatal.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.