Turkish Journal of Clinics and Laboratory

Öz

Aim: Myelodysplastic syndrome (MDS) is a hematopoietic stem cell disease characterized by bone marrow failure that usually occurs at an advanced age. The prognosis and treatment of the disease vary in patients, despite new treatments and it is still a disease that has no curative treatment rather than allogeneic stem cell transplantation. The aim of this study is to evaluate our MDS patients retrospectively in terms of demographic characteristics, prognostic score, treatments and responses of patients.
Material and Methods: Fifty six MDS patient's data was analyzed retrospectively. Demographic and diagnostic characteristics of the patients, prognostic scores, treatments, survival information were evaluated retrospectively.
Results: 25 (44%) of the patients were female, 31 (56%) were male. The median age was 63 (29-85). According to the World Health Organization (WHO) 2022 classification, 26 (47%) of the patients were classified as MDS with low blasts, 12 (21%) MDS with increased blasts 1, 7 (13%) MDS with increased blasts 2, 4 (7%) MDS with fibrosis, 3 with hypoplastic MDS, 1 MDS with low blasts and isolated 5q deletion, and 3 MDS with biallelic TP53 inactivation. 33 of the patients (59%) had at least one concomitant disease. ECOG performance status of 17 (30%) patients was found to be ≥2. According to the risk scoring, most of the patients were at high risk in the WPSS risk scoring, most of them were in the medium risk group for R-IPSS risk scores, and most of them were in the medium 1 risk group in the IPSS risk scoring.The mean duration of follow-up without treatment was 7.8 (0-95) months. As first-line treatment, 12 (22%) patients received hypomethylating agent, 13 (23%) erythropoietin stimulating treatment, 7 (12%) steroid and danazole treatment, 4 (7%) thalidomide treatment, 4 (7%) conventional chemotherapy, one patient received lenalidomide, 1 patient received antithymocyte globulin with cyclosporine. Three patients underwent allogeneic stem cell transplantation, and 11 (20%) patients were treated with supportive care without medical treatment. The first-line treatment response rate was complete response in 12 (26%) patients, partial response with transfusion reduction in 10 (23%) patients, and no response in 23 (51%) patients. A total of 7 patients had undergone stem cell transplantation when other treatment steps were included. During the follow-up, acute leukemia transformation was observed in 10 (18%) patients. At the end of the follow-up period, the number of surviving patients was 22 (40%), while 27 (48%) patients had died. The survival status was unknown for 7 patients due to the withdrawal of the follow-up. Of the 27 patients whose cause of death was known, 5 patients died due to disease progression, 12 due to infection, 2 patients due to bleeding, 6 due to transplant-related complications, and 2 due to other causes. The median total survival was found to be 38.6 (1-123) months. Age, ECOG performance status, 2016 and 2022 WHO classification, bone marrow blast ratio, bone marrow fibrosis status, IPSS-WPSS-R IPSS score, response status to treatment in treated patients, iron chelation therapy, acute leukemia transformation and allogeneic stem cell transplantation was statistically associated with survival.
Conclusion: Epidemiological data of the disease were found to be compatible with the literature in this study, in which the data of the patients monitored at our center were collected due to the introduction of a diagnostic classification update in MDS 2022 and the continuation of improvements in treatment. Together with the current classification, the distribution characteristics of the patients have been determined and will be a guide for the treatment-prognosis processes in the future. Although it is seen that a suitable treatment option is given to the majority of patients in our center, even though MDS is an advanced age disease, the responses of these treatments are not high and not permanent in accordance with the literature. The most effective factors on survival were found to be consistent with the literature. Although allogeneic stem cell transplantation, which is the only curative option for eligible MDS patients, is provided with the appropriate time and transplantation criteria, unfortunately, transplant-related mortality still seems to be a big problem. Our study will contribute to the literature in terms of providing a place for the evaluation of the new WHO classification.