The biennial International Symposium on Myelodysplastic Syndromes (MDS) was held in Washington, USA, in early May. Risk factors for MDS were among the topics discussed. Several studies were presented in which the relationships between genetic factors resp. environmental influences and MDS had been studied.
A French national study was interested in the associations between MDS and other inflammatory and autoimmune diseases (SAID) [1]. The study population consisted of 123 patients suffering from a defined SAID (vasculitis, systemic lupus erythematosus, or rheumatoid arthritis) and MDS according to international criteria. These individuals were compared in terms of characteristics and disease course with 665 patients who had MDS but no other inflammatory resp. autoimmune disease was present.
Patients with MDS and SAID were on average younger and more often men than MDS patients without SAID. MDS was diagnosed concurrently with SAID in 31% (n=38) of patients; SAID was detected earlier than MDS in 37% (n=46) and later than MDS in 32% (n=39). The only significant correlation of SAID and MDS was between systemic vasculitis and CMML. Four out of six patients with Behçet’s disease had trisomy 8; this correlation was much less frequent in patients with other SAID (four out of 79).
There were no differences in the incidence of disease progression to acute leukemia and survival rates between the two groups of patients studied.
Benzene and MDS risk
A recent analysis of case-control studies in the petroleum industry suggested that repeated benzene exposures may increase the risk of MDS [2]. Benzene is a liquid organic compound and a component of gasoline; a carcinogenic effect of benzene has been demonstrated. The possible association between low-level benzene exposure and MDS was investigated in a study from the United Kingdom. Participants were about 16,500 men who had been employed for a minimum of twelve months in one of 476 gasoline distribution centers (service stations) between 1946 and 1974. Fortunately, in the men studied, the rates of disease and death related to MDS were not higher than in the general population. Also, length of employment, period of employment, and type of employment (worker or supervisor) did not affect MDS frequency. The authors conclude that repeated exposure to low doses of benzene does not increase the risk of MDS.
Survival rates with frequent transfusions
The EUMDS registry prospectively collects data from patients in 17 countries with low-risk MDS. Most of these patients do not die from progression of the disease to acute leukemia, but due to complications as a result of bone marrow suppression. The outcome of MSD patients receiving transfusions of red cell concentrates is worse than the outcome of MDS patients without transfusions. The study authors aimed to determine whether the intensity of blood transfusions could be an independent prognostic factor for survival in MDS patients [3]. For this purpose, a study population of nearly 1000 patients who had survived the first year after MDS diagnosis and who had a good chance for a further follow-up of one year was investigated. Among other things, the intensity of blood transfusions was recorded in these patients.
During the five-year follow-up, 272 patients died: 58 after disease progression, 214 without progression. In another 34, still living patients, the disease had progressed to acute leukemia. Overall, the survival rate was 57%. Among those 806 patients who had received blood transfusions, low platelet counts were associated with more frequent blood transfusions in the next six months, and more frequent transfusions correlated with a less favorable prognosis as well as a more rapid decline in platelet counts. The best prognosis in terms of survival as well as disease progression was for those patients without blood transfusions.
Source:13th International Symposium on Myelodysplastic Syndromes, Washington, USA, April 29-May 2, 2015.
Literature:
- Mekinian A, et al: Systemic inflammatory and autoimmune diseases (SAID) associated with MDS: a french multicenter retrospective study; oral presentation 35.
- Sorahan T: Incidence of MDS in UK petroleum distribution workers; oral presentation 41.
- De Swart L, et al: Prognostic impact of transfusion intensivity on survival and thrombocytopenia in newly diagnosed lower-risk MDS patients participating in the european leukemianet MDS (EUMDS) registry; oral presentation 51.
InFo ONCOLOGY & HEMATOLOGY 2015; 3(6): 26-27.
