scholarly journals Prognostic significance of monosomal karyotype in higher risk myelodysplastic syndrome treated with azacitidine

Leukemia ◽  
2011 ◽  
Vol 25 (7) ◽  
pp. 1207-1209 ◽  
Author(s):  
R Itzykson ◽  
◽  
S Thépot ◽  
V Eclache ◽  
B Quesnel ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Prognostic Significance ◽  
Monosomal Karyotype

scholarly journals Prognostic significance of monosomal karyotype in myelodysplastic syndrome: a meta-analysis

Hematology ◽  
2018 ◽  
Vol 24 (1) ◽  
pp. 60-69 ◽  
Author(s):  
Yi-Cun Wu ◽  
Xiao-Mei Zhang ◽  
Yuan-Dong Zhu ◽  
Wei Wu
Keyword(s):  
Myelodysplastic Syndrome ◽  
Meta Analysis ◽  
Prognostic Significance ◽  
Monosomal Karyotype

313 Clinical features and prognostic significance of monosomal karyotype (MK) in the myelodysplastic syndromes (MDS)

2011 ◽  
Vol 35 ◽  
pp. S124-S125
Author(s):  
B. Nomdedeu ◽  
M. Nomdedeu ◽  
X. Calvo ◽  
M. Díaz-Bella ◽  
F. Cobo ◽  
...  
Keyword(s):  
Clinical Features ◽  
Myelodysplastic Syndromes ◽  
Prognostic Significance ◽  
Monosomal Karyotype

scholarly journals A Leukemic Stem Cell Signature-Based Scoring System for Predicting Prognosis in Myelodysplastic Syndrome Patients

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1810-1810
Author(s):  
Yu-Hung Wang ◽  
Chien-Chin Lin ◽  
Chi-Yuan Yao ◽  
Cheng-Hong Tsai ◽  
Hsin-An Hou ◽  
...  
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
High Risk ◽  
Myelodysplastic Syndrome ◽  
Scoring System ◽  
Prognostic Significance ◽  
Transformation Rate ◽  
Intermediate Risk ◽  
Hematopoietic Stem ◽  
Very High

Abstract Leukemic stem cells (LSCs) possess biological properties shared with normal hematopoietic stem cells. They are responsible for chemoresistance and relapse in acute myeloid leukemia (AML). Although myelodysplastic syndrome (MDS) has traditionally been regarded as a "hematopoietic stem cell disorder", the clinical and biological impact of LSCs on MDS patients are not well defined. To address this question, we used the Affymetrix HTA 2.0 microarray platform to profile 16 out of the 17 recently reported stemness genes (one of them, the ARHGAP22 gene was not included in our array) in our 160 adult primary MDS patients. The diagnoses were based on the 2016 World Health Organization (WHO) classification. Patients with antecedent chemotherapy or hematologic malignancies were excluded. Forty-one (25.6%) patients had MDS with single lineage dysplasia (MDS-SLD), 20 (12.5%) had MDS with ring sideroblasts (MDS-RS), 31 (19.4%) had MDS with multilineage dysplasia (MDS-MLD), 32 (20%) had MDS with excess blasts-1 (MDS-EB1) and 36 (22.5%), MDS-EB2. The risk distribution of the cohort was very-high risk, 15.3%; high risk, 21.3%; intermediate risk, 24.7%; low risk 34.7%; and very-low risk 4% according to the revised international prognosis scoring system (IPSS-R). We identified 4 genes (LAPTM4B, NGFRAP1, NYNRIN, and EMP1) whose expression levels were significantly correlated with overall survival (OS). An LSC4 score (0.731 x LAPTM4B - 1.259 x NGFRAP1 + 0.304 x NYNRIN + 0.231 x EMP1) was constructed based on the weighted sums derived from Cox regression analysis. Higher LSC4 scores were associated with higher IPSS-R scores, complex cytogenetics, and higher incidences of mutations of RUNX1, ASXL1, TP53, SRSF2 and ZRSR2. High-score patients had significantly higher 3-year AML transformation rate (36.3% vs 11.3%, P<0.001), shorter leukemia-free survival (LFS, median, 33.6 months vs not reached, P< 0.001, Figure 1A), and shorter OS (median, 15.9 months vs 77.3 months, P<0.001, Figure 1B). In subgroup analysis, LSC4 score could also well stratify MDS patients with normal karyotype into different risk groups (p<0.001). The prognostic significance of LSC4 scores for LFS, AML transformation rate, and OS still remained valid in both IPSS-R lower-risk (very low, low, and intermediate risk) subgroup (Figures 2A and 2B) and higher-risk subgroup (high and very high risk). In multivariate analysis, higher LSC4 score was an independent adverse risk factor for OS, irrespective of age, IPSS-R, and mutation status of ASXL1, TP53, EZH2, SRSF2, and ZRSR2 (Table). We also well validated the prognostic significance of the LSC4 scores in an independent cohort of 30 MDS patients (Figures 3A and 3B). Interestingly, patients with higher LSC4 scores had a better OS if they received allogeneic transplantation than those who did not. It implied that transplantation might ameliorate the poor survival impact of high LSC4 scores. In conclusion, through comprehensive analysis, we have created a simple and powerful LSC4 scoring system, which serves as an independent prognostic factor for OS and LFS in MDS patients. Disclosures No relevant conflicts of interest to declare.

Role of Paroxysmal Nocturnal Hemoglobinuria (PNH) Clones in Refractory Anemias

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4406-4406
Author(s):  
Karuna Rameshkumar ◽  
Cecil Ross ◽  
Shanthala Devi ◽  
Betsy Mathew ◽  
Arvind Kasthuri
Keyword(s):  
Flow Cytometry ◽  
Myelodysplastic Syndrome ◽  
Aplastic Anaemia ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Prognostic Significance ◽  
Laboratory Studies ◽  
Clone Size ◽  
Prospective Group ◽  
Pnh Clone

Abstract Abstract 4406 Background: The presence of paroxysmal nocturnal hemoglobinuria (PNH) clones in the setting of aplastic anemia (AA) and myelodysplastic syndrome (MDS) highlights the pathogenetic link between these disorders. The awareness that small clones of PNH cells could be detected by flow cytometry in aplastic anaemia patients who had negative Ham test has led to classifying PNH into two broad groups such as Haemolytic PNH which can have 50% presenting with thrombosis and a hypo plastic group which behaves like aplastic anaemia. Even if there is a small PNH clone in the setting of aplastic anaemia, then the patients respond better to immunosuppressive therapy. Flowcytometry is a relatively new advanced technique in many parts of India and there is no widespread consensus among methodology. Further, the clinical and prognostic significance of monitoring PNH clone size by serial flow cytometry is yet to be established here. Objective: To validate the role of flowcytometry in the diagnosis of PNH, in the setting of aplastic anaemia, myelodysplastic syndrome or abdominal or cerebral vein thrombosis in the setting of PNH which may help to prognosticate the course and treatment. Material and methods: The study was both retrospective and prospective. The prospective arm of the study was conducted from September 2009 to April 2010. Ham's test and Sucrose lysis test were done as screening tests and were reported as positive or negative. Other laboratory studies including hemogram and bone marrow analysis were done as part of standard care. In the retrospective group there were 32 patients who were diagnosed as PNH based on Ham's and Sucrose lysis test followed up from 1990. Four color flowcytometry immunophenotypic analysis with CD55 and CD59 was used to detect PNH clones in RBCs. EDTA-anticoagulated whole blood was stained with anti–CD 55-Phycoerythrin (PE) and anti–CD 59-PE.A minimum of 10,000 events were acquired. For analysis, RBCs were identified by light-scatter properties. For the purpose of the study, 3% of type II and/or type III cells were considered as threshold to diagnose PNH clones in both CD55 and CD 59 gated population. The results of laboratory studies including transfusion history and flow cytometry were correlated. Institutional ethical committee approval was obtained for the conduct of the study. Results: 33 patients were included as part of the prospective group. The number of males was more compared to females (19:14). 78% of patients had hemoglobin of less than 7gm%. The presentation varied from unexplained anemia to thrombosis. Based on flow cytometry, 3 patients were found to have PNH clones in the RBC lineage by both CD 55 and CD 59. CD 55 alone picked up one more patient. In the retrospective group there were 32 patients who were followed up from 1990. Interestingly, two of them who had earlier presented with aplastic anemia had transformed into PNH in a span of 3years and 6 months. All patients were treated with Stanazolol and cyclosporine. The median survival was 12.14. months with Kaplan-Meier survival estimates of 30 and 20 percent at 121 months, and 143 months after diagnosis, respectively. The patients in the prospective group are being followed up. Discussion: The Ham test, sucrose lysis test, and modified Ham tests rely on the differential sensitivity of PNH red cells to haemolysis. These tests, although suitable for haemolytic PNH, cannot reliably detect small populations of PNH red cells nor differentiate partially and completely deficient cells. One patient who presented with DVT in whom increase in clone size was demonstrated by serial flowcytometry. Testing for the presence of PNH RBC clones provides additional diagnostic information and the thrombotic event may probably directly related to the size of the PNH clone. Conclusions: Our data demonstrate the utility of RBC assay in flow cytometry in the primary screening of PNH, AA, and MDS patients. The clinical and prognostic significance of monitoring PNH clone size by serial flow cytometry is relatively new and could be demonstrated in one patient. Disclosures: No relevant conflicts of interest to declare.

Prognostic significance of deletion of the long arm of chromosome 20 in patients with myelodysplastic syndrome (MDS): a study of the Greek MDS Study Group

2006 ◽  
Vol 0 (0) ◽  
pp. 060928002456003-???
Author(s):  
Athanasios G. Galanopoulos ◽  
Argyris Symeonidis ◽  
Aleka Kourakli ◽  
Eleni A Papadaki ◽  
Panagiotis Tsaftaridis ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Prognostic Significance ◽  
Study Group ◽  
Chromosome 20
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