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Use of Hematopoietic Stem Cell Transplantation to Assess the Origin of Myelodysplastic Syndrome
Published on: October 3, 2018
Proliferative indices, cytogenetics, immunophenotye and other prognostic parameters in myelodysplastic syndromes
1Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. varmaneelam@yahoo.com
Abstract:
Thirty-five adult myelodysplastic syndrome (MDS) patients were included in this study: 11 refractory anemia (RA), 4 RA with ring sideroblasts (RARS), 9 RA with excess of blasts (RAEB), 10 RAEB in transformation (RAEB-T) and 1 chronic myelomonocytic leukemia (CMML). The ranges of survival were 4-51 months, 40-59 months, 7-38 months, 5-24 months and 5 days, respectively. Three patients died and 3 showed disease progression during the course of the study. A composite analysis of proliferative indices, cytogenetics, immunophenotype and other conventional/novel prognostic parameters in the context of Indian MDS patients was performed. The proliferative indices (AgNOR and Ki 67 positivity), immunophenotypic markers, serum LDH and ferritin levels revealed wide variations and great overlap among different FAB subtypes. The scoring systems (Bournemouth, Dusseldorf and Goasguen) did not correlate with the prognosis and survival (p > 0.05). Clonal cytogenetic abnormalities were detected in 24/35 (68.57%) patients, +8, -5 and -7 being observed commonly. Cytogenetic abnormalities were more frequent in RAEB (88.8%), RAEB-T (80.0%) and RA (63.6%) subtypes of MDS. By Using Mufti's prognostic system and International prognostic scoring system (IPSS), a good positive correlation was found between low risk category and RARS with better survival as compared to other risk categories/FAB subtypes (p < 0.01). However, rest of the FAB subtypes were assigned into high, intermediate and low risk categories without any correlation with the survival and/or leukemic transformation. RARS subtype revealed itself as the better prognostic category according to the cytogenetic findings as well as Mufti's grading system. This was possible due to the longer follow up available for these patients (40-59 months).
Insights
This study analyzed prognostic factors in 35 myelodysplastic syndrome (MDS) patients. Refractory anemia with ring sideroblasts (RARS) showed better survival and prognosis compared to other subtypes.
Area of Science:
- Hematology
- Oncology
- Clinical Pathology
Background:
- Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic stem cell disorders.
- Accurate prognostication is crucial for managing MDS patients and guiding treatment decisions.
- Existing prognostic scoring systems may require validation in diverse patient populations, such as those in India.
Purpose of the Study:
- To evaluate various prognostic parameters in Indian adult patients with myelodysplastic syndrome (MDS).
- To assess the correlation between different FAB subtypes, proliferative indices, immunophenotype, cytogenetics, and survival outcomes.
- To compare the efficacy of established scoring systems (Bournemouth, Dusseldorf, Goasguen, Mufti's, IPSS) in predicting prognosis for Indian MDS patients.
Main Methods:
- A cohort of 35 adult MDS patients across different FAB subtypes (RA, RARS, RAEB, RAEB-T, CMML) was studied.
- Analysis included proliferative indices (AgNOR, Ki 67), immunophenotypic markers, serum LDH and ferritin, and cytogenetics.
- Prognostic scoring systems (Bournemouth, Dusseldorf, Goasguen, Mufti's, IPSS) were applied and correlated with survival and disease progression.
Main Results:
- Wide variations and overlaps in proliferative indices and immunophenotypic markers were observed among FAB subtypes.
- Clonal cytogenetic abnormalities were detected in 68.57% of patients, with +8, -5, and -7 being common.
- Mufti's prognostic system and IPSS showed a positive correlation between low-risk categories, particularly RARS, and better survival (p < 0.01).
- Other scoring systems (Bournemouth, Dusseldorf, Goasguen) did not correlate with prognosis or survival (p > 0.05).
- The RARS subtype demonstrated a better prognostic profile based on cytogenetics and Mufti's grading system.
Conclusions:
- The refractory anemia with ring sideroblasts (RARS) subtype of MDS exhibits a favorable prognosis in the Indian population.
- Cytogenetic findings and Mufti's grading system are valuable in assessing prognosis for RARS.
- Standard prognostic scoring systems may need refinement or validation for specific ethnic and geographic cohorts to accurately predict survival and leukemic transformation in MDS.

