scholarly journals Refractory cytopenia with multilineage dysplasia

2020 ◽  
Author(s):  
Keyword(s):  
Multilineage Dysplasia

scholarly journals Heterogeneity of Mesenchymal Stromal Cells in Myelodysplastic Syndrome-with Multilineage Dysplasia (MDS-MLD)

2019 ◽  
Vol 35 (2) ◽  
pp. 223-232 ◽  
Author(s):  
Salar Abbas ◽  
Sanjay Kumar ◽  
Vivi M. Srivastava ◽  
Marie Therese M. ◽  
Sukesh C. Nair ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Mesenchymal Stromal Cells ◽  
Stromal Cells ◽  
Multilineage Dysplasia

25 Refractory anemia, pure sideroblastic anemia and refractory cytopenia with multilineage dysplasia

1997 ◽  
Vol 21 (1) ◽  
pp. S7
Author(s):  
G. Pérez Rus ◽  
J. del Toro ◽  
T. Calabuig ◽  
J. Anguita ◽  
J. Sánchez Fayos
Keyword(s):  
Refractory Anemia ◽  
Sideroblastic Anemia ◽  
Multilineage Dysplasia

Clinical Relevance of Bone Marrow Fibrosis and CD34-Positive Cell Clusters in Primary Myelodysplastic Syndromes

2009 ◽  
Vol 27 (5) ◽  
pp. 754-762 ◽  
Author(s):  
Matteo Giovanni Della Porta ◽  
Luca Malcovati ◽  
Emanuela Boveri ◽  
Erica Travaglino ◽  
Daniela Pietra ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Overall Survival ◽  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
Transfusion Requirement ◽  
Free Survival ◽  
Cell Clusters ◽  
Multilineage Dysplasia ◽  
Poor Risk ◽  
Marrow Fibrosis

Purpose We studied bone marrow (BM) histologic abnormalities in myelodysplastic syndromes (MDS) classified according to WHO criteria to determine their clinical correlates and prognostic value. Patients and Methods Three hundred one consecutive patients were retrospectively evaluated for BM fibrosis and CD34 immunoreactivity. Marrow fibrosis was assessed following the European consensus guidelines. Results Moderate to severe BM fibrosis was detected in 17% of cases and was associated with multilineage dysplasia (P = .001), high transfusion requirement (P < .001), and poor-risk cytogenetics (P = .007). CD34+ cell clusters were found in 23% of patients and were associated with WHO categories with excess of blasts (P < .001) and poor-risk cytogenetics (P = .001). In multivariable analysis, BM fibrosis and presence of CD34+ cell clusters had independent negative impact on overall survival (P < .001 and P = .019, respectively) and leukemia-free survival (P < .001 and P = .004, respectively). A hierarchical clustering analysis identified three subsets of patients with distinct clinical features. One cluster consisted mainly of patients with BM fibrosis, multilineage dysplasia, and high transfusion requirement; these individuals had lower overall survival and leukemia-free survival (P = .001 and P < .001, respectively). Within patients stratified according to International Prognostic Scoring System and WHO classification–based Prognostic Scoring System categories, BM fibrosis involved a shift to a one-step more advanced risk group. Conclusion BM fibrosis identifies a distinct subgroup of MDS with multilineage dysplasia, high transfusion requirement, and poor prognosis and represents an independent prognostic factor that may be useful in clinical decision making. Furthermore, the presence of CD34+ cell clusters is an independent risk factor for progression to acute leukemia.

Overexpression of CXCR4 in Acute Myeloid Leukemia Predicts Adverse Overall and Progression-Free Survival Independently of FLT3 Gene Mutation.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1890-1890
Author(s):  
Sergej Konoplev ◽  
George Z. Rassidakis ◽  
Elihu H. Estey ◽  
Hagop M. Kantarjian ◽  
Xuelin Huang ◽  
...  
Keyword(s):  
Gene Mutation ◽  
Creatinine Level ◽  
Myeloid Leukemia ◽  
Performance Status ◽  
Cxcr4 Expression ◽  
Free Survival ◽  
Prior Chemotherapy ◽  
Multilineage Dysplasia ◽  
History Of ◽  
Acute Myeloid

Abstract CXC chemokine receptor 4 (CXCR4) expression in acute myeloid leukemia (AML) is reported to correlate with FLT3 gene mutation and poorer prognosis. We analyzed the prognostic significance of CXCR4 expression in 122 AML patients with diploid karyotype and no evidence of FLT3 gene mutation treated at our institution between 1997 and 2003. All patients received intensive chemotherapy according to institutional protocols; 84% received cytarabine-containing regimens. Bone marrow biopsy specimens obtained at diagnosis from untreated patients were immunostained with an antibody specific for CXCR4 (Abcam, clone 2074; Cambridge, MA). Statistical analysis included the following variables: CXCR4 expression, age, sex, race, incorporation of cytarabine in the therapeutic regimen, presence of multilineage dysplasia, history of antecedent hematologic disorder, history of prior chemotherapy (for either hematological or non-hematological disorder), performance status, leukocytosis, hemoglobin, platelet count, bilirubin, and creatinine. There were 70 men and 52 women with a median age of 62 years (range, 22–82 years). Median follow-up was 18 months (range, <1–97 months). Seventy-six patients achieved complete remission (CR); 39 relapsed. Sixty-six patients died, including 9 with no evidence of disease. CXCR4 was positive in 70 and negative in 52 patients, with CR rates of 58% and 71%, respectively (p=0.09). In the univariate Cox model, CXCR4 expression was associated with shorter overall survival (OS), p=0.008, and disease-free survival (DFS), p=0.012, figure1. Figure Figure Shorter OS was also associated with failure to achieve CR (p=<0.0001), age (p=0.01), presence of multilineage dysplasia (p=0.006), poorer performance status (p=0.049), higher creatinine level (p=0.00002), and lower hemoglobin level (p=0.042). Shorter DFS was associated with failure to achieve CR (p=0.0002), presence of multilineage dysplasia (p=0.02), history of prior chemotherapy (p=0.04), and higher creatinine level (p=0.0009). In multivariate analysis, shorter OS was associated with CXCR4 expression (p=0.047), age (p=0.029), presence of multilineage dysplasia (p=0.042), and high creatinine level (p=0.016). Shorter DFS was associated with CXCR4 expression (p=0.036), history of prior chemotherapy (p=0.044), presence of multilineage dysplasia (p=0.007), and high creatinine level (p=0.034). These results suggest that CXCR4 expression is associated with poor prognosis in AML patients and is independent of FLT3 gene mutation.

The Detection of Multilineage Dysplasia (MLD) Has No Influence on Prognosis in NPM1 Mutated Acute Myeloid Leukemia (AML) with Normal Karyotype

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2518-2518
Author(s):  
Ulrike Bacher ◽  
Susanne Schnittger ◽  
Wolfgang Kern ◽  
Tamara Weiss ◽  
Claudia Haferlach ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Normal Karyotype ◽  
Biological Entity ◽  
Prognostic Impact ◽  
Npm1 Mutation ◽  
Cox Regression Analysis ◽  
Multilineage Dysplasia ◽  
Acute Myeloid

Abstract Acute myeloid leukemia with mutated nucleophosmin (AML NPM1mut) represents about one-third of all adult AML and shows distinctive biological and clinical features. For this reason, AML NPM1mut is planned to be included as a separate category in the revised WHO classification. A yet controversial issue, however, is whether AML NPM1mut with or without multilineage dysplasia (MLD) may differ biologically and clinically, as the presence of MLD might confer a negative prognostic impact. A further feature that was suggested to be typical for NPM1 mutated AML is “cup-like” morphology of blasts. We here analyzed 128 pts with AML NPM1mut and normal karyotype at first manifestation (59 females, 69 males; median age 60.5 years; 23.5–79.3 y). We investigated in parallel cytomorphology from bone marrow and/or peripheral blood, chromosome banding analysis, and molecular analyses. Presence of dysplasia was defined by dysplastic features in ≥50% of cells in the respective hematopoietic lineage as defined by the WHO. A 5% cut-off was taken for the presence of “cup-like” morphology of blasts. All cases were additionally analyzed for the FLT3-ITD, and in 122 pts for the FLT3-TKD. Statistical analysis was performed for overall survival (OS), and event-free survival (EFS) according to Kaplan-Meier using the 2-sided log-rank test. Cox regression analysis related OS and EFS with the analyzed parameters. We found a predominance of the FAB M1 (21.3% of all cases), M2 (33.9%), and M4 subtypes (28.3%). Cup-like morphology in ≥5% of all blasts was observed in 39 of 127 evaluable cases (31.3%) confirming previous observations of an association of the NPM1mut and this specific blast appearance. Molecular characterization detected NPM1 mutation subtype A (n=90/122; 73.8%), B (15/122; 12.3%), and D (7/122; 5.7%), which was in accordance to previous studies. In 56 cases (43.8%) there was a coincidence with an FLT3-ITD. Dysplasia of granulopoiesis was detected in 28/126 (22.2%), of erythropoiesis in 28/104 (26.9%), and of megakaryopoiesis in 57/87 (44.5%) cases in which the respective cell lineage could be analyzed. MLD (≥2 dysplastic hematopoietic lineages) was detected in 28 of 105 evaluable cases (21.9%). Clinical follow-up was available in 104 pts. (median follow-up 12,7 months). CR rate was 83.1% in 77 evaluable pts., and median EFS was 42.1 months in 104 evaluable pts (median OS not reached). An additional FLT3-ITD had a significantly inferior OS (p=0.003) and EFS (p=0.007), confirming the present series being representative. However, the presence of MLD was not significantly related to any endpoint such as CR rate, EFS, or OS. There was no association between MLD and the NPM1-subtype. Also, there was no significant correlation of MLD and the presence of a FLT3-ITD. In conclusion, the presence of MLD in AML NPM1mut with normal karyotype had no impact on CR rate and outcome, whereas coincidence of FLT3-ITD significantly worsened prognosis. These results give further evidence that AML with NPM1mut AML is a unique biological entity with clinical course mainly influenced by FLT3-ITD coincidence. These data do not support any additional prognostic influence of MLD in this AML subtype.

Independent Validation of the 2008 World Health Organization (WHO) Reclassification of Myelodysplastic Syndromes (MDS) Associated with Ring Sideroblasts

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2686-2686 ◽  
Author(s):  
David P. Steensma ◽  
Curtis A Hanson ◽  
Ayalew Tefferi
Keyword(s):  
High Risk ◽  
Median Survival ◽  
Scoring System ◽  
Who Classification ◽  
Risk Group ◽  
Low Risk ◽  
Risk Category ◽  
Intermediate Risk ◽  
Multilineage Dysplasia ◽  
Ring Sideroblasts

Abstract Background: The 2001 WHO classification of myeloid neoplasms distinguished 2 forms of MDS associated with &gt;=15% ring sideroblasts and &lt;5% marrow blasts: refractory cytopenia with multilineage dysplasia and with ring sideroblasts (RCMD-RS) vs. refractory anemia with ring siderblasts (RARS, erythroid-restricted dysplasia). However, the real prognostic value of separating RCMD-RS from RCMD with &lt;15% ring sideroblasts and from RARS is uncertain, and the WHO has proposed merging RCMD-RS and RCMD in the 2008 classification revision. Furthermore, the WHO-based Prognostic Scoring System (WPSS), proposed by Malcovati and colleagues in 2005 as a dynamic system that overcomes some of the limitations of the 1997 International Prognostic Scoring System (IPSS), has undergone limited independent external validation to date and its applicability to sideroblastic MDS in particular is unclear. We assessed the validity of the 2008 WHO reclassification and the WPSS for MDS cases associated with &gt;=15% ring sideroblasts and a normal blast proportion. Methods: We reviewed WPSS and IPSS component parameters at diagnosis and the clinical outcomes of 465 patients (68% males, median age 72) evaluated at our institution over a 13-year period: 140 with RARS, 114 with RCMD-RS, and 211 with RCMD. Patients were assigned a WPSS score and risk category (very low-risk group=0 points; low=1; intermediate=2, high=3 or 4) by summing 3 subscores: 2001 WHO classification (0 for RARS, 1 point for RCMD or RCMD-RS), IPSS cytogenetic risk group (0=good, 1=indeterminate, 2=poor), and red cell transfusion dependence (0=no, 1=yes). Survival was assessed by Kaplan-Meier estimates, and prognostic factors examined by proportional hazards analysis. Results: The median time until death or last followup was 26 months, and 70% of patients were known to have died. The median survival by WHO MDS subtype was 75 months for RARS, 25 months for RCMD-RS, and 26 months for RCMD (Log-Rank p&lt;0.0001 for RARS vs. either RCMD-RS or RCMD; p=0.60 for RCMD vs. RCMD-RS ). Both the WPSS and IPSS predicted overall survival in patients with ring sideroblasts. Median survival for the patients grouped by WPSS risk category was 89 months for very low risk (n=95), 41 for low risk (n=198), 31 for intermediate risk (n=82), and 11 for high risk (n=91) (p&lt;0.0001, except for low risk vs. intermediate risk, p=0.31). (Very high risk WPSS scores cannot be achieved without excess marrow blasts, and such patients were excluded from this analysis.) Median survival by IPSS was 73 months for low-risk, 33 months for intermediate-1, and 8 months for intermediate 2 (p&lt;0.0001). The IPSS’ predictive power was unchanged if patients with secondary MDS were included or excluded (the IPSS was based on a review of 816 patients with apparently de novo MDS). Conclusions: These data support the WHO’s proposal to merge RCMD and RCMD-RS, and suggest that the adverse prognostic significance of multilineage dysplasia renders the presence of ring sideroblasts unimportant. The WPSS is a valid prognostic tool in patients with MDS associated with ring sideroblasts, but in this subgroup both the WPSS and IPSS stratify patients into 3 risk groups, and the WPSS does not offer additional value over the IPSS. Figure Figure

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