Sociodemographic Characteristics Of Survival In Patients With Myelodysplastic Syndrome

Myelodysplastic syndrome (MDS) is a heterogeneous group of acquired hematopoietic stem cell disоrders characterized by ineffective hematopoiesis and a high risk of transformation into acute myeloid leukemia (AML). Due to the phenotypic diversity of MDS, survival widely varies. The purpose of the present study is to analyze the impact of some socio-demographic characteristics on the survival of patients with MDS. We analyzed 219 patients with MDS, who were admitted in the Clinic of Hematology, University Hospital "St. Marina”- Varna for a period of 10 years (2010-2020). Survival was assessed by age, sex, FAB and WHO2016 subtype and risk group defined by IPSS, IPSS-R and WPSS. There is a significantly higher survival rate in women and an inversely proportional relationship between survival and age. Patients with RAEB and RAEB-t have the lowest survival as well as patients with high and very high risk. MDS presents with significant differences in survival between subtypes, age and sex. The outcome of the disease varies according to the risk group determined by the established scales for risk stratification, and the most accurate in the prognosis is IPSS-R

Decreased periferal blood dendritic cells in multiple Myeloma: The potential role of IL-6 and Beta-2-Microglobulin

Introduction: Several studies demonstrate the presence of quantitative and functional abnormalities in the dendritic cell (DC) subsets in patients with multiple myeloma (MM). The inhibitory effect of IL-6, TGF-!, IL-10 and beta-2-microglobuline (beta-2-MG) is highly suspected.Purpose: The aim of the study was to evaluate the myeloid and plasmacytoid dendritic cells (MDC and PDC) in newly diagnosed patients with MM in correlation with various biological markers.Materials and Methods: Thirty patients with newly diagnosed MM were included in the study. All the laboratory parameters were obtained at the time of diagnosis. Three colour flow cytometry with ILT3/lin/CD11c was used for the detection of the two peripheral blood dendritic cell (PBDC) subsets. The plasma level of IL6 was detected by ELISA (Standard Curve Range: 2-200pg/ml); the beta-2-MG- by the immunoturbidimetric test.Results: The median age of patients was 61.5 years (36-89). The mean M-protein concentration was 46.5±16.2 g/l. IgG kappa was detected in 15 patients, IgG lambda-in 4, IgA kappa-in 7, IgA lambda-in 3. The mean level of beta-2-MG was 7.0±5.7mg/l (1.82-22.49 mg/l ); beta-2-MG was used to determine the stage according to the ISS. The mean level of IL6 was 27.73±21.47pg/ml (4.6-72.5 pg/ml). The percentage of MDC and PDC was significantly lower in the periferal blood of patients with MM in comparison to healthy subjects (0.08%±0.09% vs 0.21%±0.02% and 0.04%±0.03% vs 0.16%±0.01%, respectively). A statistically significant difference was found between the percentage of MDC and PDC in the different stages. There was a negative correlation between MDC and PDC and the levels of beta-2-MG (p=0.02 and p=0.02), as well as between MDC and the IL6 levels (p=0.04). No correlation was found between MDC, PDC, levels of M-protein and the type of paraprotein.Conclusion: Our results demonstrate the relationship between peripheral blood DC, IL6 and beta-2-MG and confirm the published data for the inhibitory effect of the two factors on DC differentiation and maturation in vitro. The monitoring of beta-2-MG and IL6 may have clinical implication as a predictor of the immune system status as well as for the yield of harvested DCs for vaccination

Cell adhesion molecules in pleural effusions with different etiology

The pleura, including the mesothelial and underlying mesenchymal cells and extracellular matrix, is often involved in pathological processes of not completely defined mechanisms. Pleural cells are specialized in performing barrier and secretory functions and require careful study to gather a meaningful clinical information.Biomedical Reviews 1996; 6: 121-123

Severity of clinical manifestation, and prognosis of patients with myelofibrosis

Introduction: Primary myelofibrosis (PMF) is a myeloproliferative disorder characterized by bone marrow fibrosis and ineffective extramedullary hematopoiesis, which presents with anemia, constitutional symptoms, and excessive splenomegaly. A number of factors are involved in the pathogenesis of the anemia in PMF, including impaired iron metabolism regulation. It was found that higher levels of the key regulator of iron metabolism - the peptide hormone hepcidin in patients with PMF, are associated with the severity of the anemia, blood transfusion dependence and decreased overall survival.Aim: The aim of the study was to analyze the serum levels of hepcidin in patients with PMF and its impact on the clinical course, prognosis, and outcome of the disease.Materials and Methods: A total of 68 patients with PMF and 12 healthy controls were analyzed. Serum hepcidin levels were measured by ELISA.  The results were statistically analyzed by dispersion, comparison, and correlation methods.Results: Then mean hepcidin levels in patients with PMF were statistically significantly higher compared to healthy controls. (99.05 ng/mL; 20.57 ng/mL; F = 7.95; p = 0.006). High levels of hepcidin correlated with high risk according to DIPSS (p = 0.046), carrier of JakV617F mutation (p = 0.022), fibrotic phase according to WHO 2016 (p = 0.062), and the number of blood transfusions per month (p = 0.005). Higher hepcidin levels were not relevant to overall survival.Conclusion: Hepcidin is a biological marker the monitoring of which in the course of MF would help for a more accurate clinical and prognostic assessment of the disease

Outcome after azacitidine treatment in patients with high-risk myelodysplastic syndrome and acute myeloid leukemia in the Clinic of Hematology at St. Marina University Hospital, Varna

Introduction: Hypomethylating agents have become a standard therapy for high-risk myelodysplastic syndromes (MDS) and elderly patients with acute myeloid leukemia (AML).Aim: The aim of the study was to assess the efficacy of azacitidine treatment in patients with MDS and AML followed for 18 months.Materials and Methods: Twenty-seven patients with MDS and AML treated in the Clinic of Hematology at St. Marina University Hospital, Varna were included in the study. Azacitidine was administered subcutaneously in at a dose of 75 mg/m2 for 7 days. Disease assessment was performed on  the 3rd month, 6th month, and at progression.Results: Twenty-seven patients were analyzed. Their median age was 71.5 years. Nine had refractory anemia with excess of blasts II (RAEB II), 5 had chronic myelomonocytic leukemia II (CMML II), 1 was with unclassifiable MDS (MDS-U), and 12 with AML. The median number of administered cycles was 6 (1-19). Eleven patients completed 6 cycles of azacitidine. Partial response was achieved in 9 patients (33%) (7 MDS and 2 AML), stable disease in 8 (29%) (5 MDS and 3 AML). Progressive disease was observed in 10 patients (37%). The response correlated with the type of the disease (p=0.03), cytogenetic risk (p=0.01), and survival (p=0.000). At 18 months, 60% of MDS patients were alive compared to 41.7% in the AML group. The median time to death in the AML patient group was 2.5 months. The mean overall survival was 10.4 months (12.6 months for MDS patients and 5.4 months for AML patients).Conclusion: The therapy with azacitidine is an option for elderly patients with high-risk MDS.  In patients with AML a rapid progression is observed during the first two cycles with mortality rate of 58.3%

Novel approaches in the classification and risk assessment of patients with myelodysplastic syndromes-clinical implication

INTRODUCTION: New prognostic systems have been proposed aimed at improving the ability to predict survival and progression in myelodysplastic syndome (MDS) patients, as well as to select the accurate therapy.PURPOSE: The aim of this study was to determine the prognostic score for patients with MDS, diagnosed in the Hematology Clinic, University hospital, Varna, comparing different prognostic scoring systems.MATERIALS AND METHODS: 92 patients with MDS, diagnosed between 2004 and 2012 were included in the study. The mediana of age was 72 (20-91 years), 87% >60 years of age, 52 men and 41 women. The prognostic score was determined according to IPSS, WPSS, MDACSS. The parameters assessed were WHO type, karyotype, cytopenias, percentage of marrow blasts, age, performing status and transfusion dependence.RESULTS: Cytogenetic studies were performed in 100% of patients. Abnormalities were found In 34.8%, the most common-del-5q (14.6%), -Y (4.5%), complex karyotype (8%), +8 (2.2%). Patients were distributed according to the cytogenetic risk -low-87.2%, intermediate-7.7%, high-5.1%. According to IPSS 41% of the patients were with low risk, 34%-Int-1, 16%-Int-2, high-9%.  WPSS distributed the patients in 5 groups: very low-11%, low risk-43%, int-21%, high-20%, very high-5%. According to MDACSS 14% of the patients were with low, 54%-Int-1, 18%-Int2, 13% with high risk.CONCLUSION: Significant correlation was found between the risk groups and AML transformation and survival. However, the comparison between the different scoring systems demonstrated the advantage of prognostic systems, based on broader range of clinical parameters in better distribution of intermediate risk patients and the precise determination of high and very high risk.

18F-FDG PET/CT in the diagnosis of an extranodal relapse of diffuse large B-cell lymphoma (DLBCL): a clinical case with a literature review

Extranodal lymphoma, secondary to or accompanying nodal disease is uncommon, but not unusual finding. 18-Fluorodeoxyglucose positron emission tomography (18F-FDG PET/CT) imaging has an essential role in the staging of lymphoma, in treatment response monitoring, and in detection of recurrence. We present a case of a 52-year-old man with generalized diffuse large B-cell lymphoma (DLBCL) with multiple extranodal sites involvement detected by 18F-FDG PET/CT. With this clinical case we demonstrate that 18F-FDG PET-CT is a more effective technique than CE-CT for the evaluation of viable extranodal involvement of the diffuse large B-cell lymphoma (DLBCL) and should be combined in the monitoring of DLBCL

The role of MDS-CI in the prognostic assessment of patients with myelodysplastic syndrome

Myelodysplastic syndrome (MDS) is a heterogeneous group of acquired hematopoietic stem cell disorders characterized by ineffective hematopoiesis and a high risk of transformation into acute myeloid leukemia (AML). The MDS comorbidity index (MDS-CI) is designed to predict the impact of comorbidities on the outcome of the disease. Тhe aim of our analysis is to assess the prognostic value of MDS-CI within the WHO prognostic scoring system (WPSS) subgroups. We applied MDS-CI in 219 patients with MDS, diagnosed and treated in the Clinic of Hematology of St. Marina University Hospital, Varna, Bulgaria between May 2010 and May 2020. WPSS was used for prognostic stratification. Statistical analysis was performed using SPSS 20. We found that the mean age of patients with MDS was 70.7 ± 10.2 years (35–93 years). In patients with very low/low risk according to WPSS, we found significant difference in terms of survival between MDS-CI = 0 and MDS-CI > 2 (69.2 ± 43.0 vs. 38.3 ± 42.1 months, p < 0.001). Similar difference was found within the intermediate/very high risk groups (p < 0.001). MDS-CI adds prognostic value to the established WPSS. Combining both systems allows refining the prognostic assessment and survival of MDS patients

Therapeutic approach in the treatment of newly diagnosed elderly patients with secondary acute myeloid leukemia—a clinical case and review of the literature

Acute myeloid leukemia (AML) is a therapeutic challenge in elderly patients because of the biology of the leukemia and the poor functional status of the patient. Acute myeloid leukemia following previous treatment (t-AML) is defined as a separate subtype of AML, which is associated with the late effects of previous chemotherapy or radiotherapy. The therapeutic results of patients with t-AML are limited and optimal treatment in these patients is an even greater challenge.Our patient is a 68-year-old woman who was hospitalized due to isolated anemia. The patient was diagnosed with breast cancer in 1996 and ovarian cancer in 2014, which were in remission at the time of the present hospitalization. Diagnostic procedures revealed therapy-related myelodysplastic syndrome (t-MDS) with a complex karyotype. The patient was stratified as high risk according to the revised IPSS. The patient received the best supportive care. Two months later, she was re-hospitalized due to fever, progressive weakness and fatigue, stomach discomfort, loss of appetite. Laboratory tests showed pancytopenia with 25% of myeloblasts in peripheral blood. The reassessment of the disease revealed evolution to t-AML with MDS-associated changes. Treatment with a hypomethylating agent was initiated. Unfortunately, the patient died with the symptoms of sudden cardiac death.The therapeutic decision in elderly patients with t-AML is difficult and multifactorial. Combinations of already approved agents and new molecules will improve and diversify the therapeutic choices in elderly patients with high-risk AML