A comparative review of classification systems in myelodysplastic syndromes (MDS).

James P. Wilmot Cancer Center, University of Rochester Medical Center, Rochester, NY 14642, USA.
Seminars in Oncology (Impact Factor: 3.94). 09/2005; 32(4 Suppl 5):S3-10. DOI: 10.1053/j.seminoncol.2005.06.021
Source: PubMed

ABSTRACT Reliable classification and prognostic scoring systems for myelodysplastic syndromes (MDS) are needed to facilitate medically appropriate treatment and management decisions. The French-American-British (FAB) classification scheme for MDS was described in 1982 and has become the reference standard for subsequent MDS classification schemes. The FAB classification system divides MDS into five subgroups, based mainly on morphologic criteria and the percentage of myeloblasts in bone marrow (BM). More recently, the increasing availability of BM cytogenetics, immunologic markers, and molecular genetics has provided important information for staging, prognosis, and treatment of MDS. A World Health Organization panel incorporated this new diagnostic information into a revised classification system that modified the FAB criteria while retaining most of its basic features. The major changes included the creation of additional categories (eg, 5q- syndrome), distinction of unilineage from multilineage dysplasias in the refractory anemias, and subdivision of the heterogeneous refractory anemias with excess blasts into two categories based on BM blast percentage. Additionally, some MDS subtypes were removed or merged with other myeloid disorders into newly created categories. In independent validations, the World Health Organization revisions were shown to provide more-homogeneous subgroups of patients and greater prognostic power compared with the FAB system, although controversies remain. The International Prognostic Scoring System combines blast percentage, karyotype, and number of cytopenias to generate a scoring system that reliably estimates survival and risk of transformation to acute myeloid leukemia for patients with MDS. This universally accepted scoring system is often combined with FAB or World Health Organization morphologic criteria to provide a more complete clinical picture and the most accurate prognostic assessment possible. As more is learned about the pathogenesis of MDS at the molecular level, it is anticipated that these classification and scoring systems will continue to evolve to incorporate the new information.

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: antigen expression pattern in refractory anemia, refractory anemia with ringed sideroblasts and refractory anemia with excess blast cases using flow cytometry. Journal of Medicine and Biomedical Sciences 2013; 4(2), 49-56. DOI: 10.7813/jmbs.2013/4-2/8 ABSTRACT Myelodysplastic Syndromes (MDSs) are a slow progressing group of disease that causes peripheral cytopaenia of all cell lineages, with high capabilities to transform into acute myeloid leaukaemia (AML). Establishing a frame of reference for antigen expression pattern in different MDS subtypes, may help to classify the patients for choosing more appropriate therapeutic approaches. In this study, thirty bone marrow (BM) samples from newly-diagnosed MDS patients were analyzed by 4-colour FACS Canto flow cytometer. In this research, the quantitative analysis of antigen expression patterns of granulocytic, monocytic, erythroid and lymphoid lineages also myeloid precursors were performed. The one-way ANOVA was used to test the differences between mean percentages of antigens in MDS subtypes. In this study, we showed the mean percentages of CD71/CD235a/CD45/CD117, HLA-DR/CD13/CD45/CD11b, CD14/CD33/CD45/CD34, and CD19/ CD20/CD45/CD10 antibody combinations on erythroid, granulocytic, monocytic, and lymphoid lineages, respectively, in different MDS subtypes. The most important finding of this study was the significant difference of CD71 mean percentage in erythroid cells between MDS subtypes. Erythroid lineage in Refractory Anemia with Excess Blast (RAEB) cases was found by lower mean percentage of CD71 (54.74%), as compared to Refractory Anemia (RA) (74.65%) and Refractory Anemia with Ringed Sideroblasts (RARS) cases (74.60%). In this study, the difference of CD71 + erythroid cells was statistically significant only between RARS and RAEB subtypes (p=0.011). Our study, showed the expression range of different CD markers on different lineages of MDS subtypes. These findings can improve understanding the prognosis of various subtypes, and explaining the laboratory and clinical results.
    Mohadese H. Boroojerdi, N. Daneshvar, P. Ghoraishizadeh, S. Md Noor, Z. Seman, R. Ramasamy. Descriptive analysis of antigen expression pattern in refractory anemia, refractory anemia with ringed sideroblasts and refractory anemia with excess blast cases u; 04/2013
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background: Myelodysplastic syndrome is a mixed clonal disorder of bone marrow progenitor cells. Understanding the pattern of the different lineage-specific, immature, and mature markers in myelodysplastic syndrome will help in setting-up the frame of reference to diagnose. Patients and Methods: We compared 60 bone marrow samples from 30 newly-diagnosed patients with myelodysplastic syndrome and 30 patients with idiopathic thrombocytopenic purpura as the control to perform a quantitative analysis of the antigen expression patterns in granulocytic, monocytic, erythroid and lymphoid lineages and myeloid precursors. Results: Quantitative analysis of CD markers, showed that the mean percentages of CD33, CD13, CD11b, HLA-DR, CD10 and CD34 positive granulocytes were 91%, 84.98%, 77.20%, 14.59%, 40.34% and 34.25%, respectively in myelodysplastic syndrome and 96.89%, 91.57%, 81.47%, 10.56%, 58.30% and 32.37%, respectively in idiopathic thrombocytopenic purpura. Flow cytometric analysis of erythroid lineage showed the mean percentage of CD71 in myelodysplastic syndrome and idiopathic thrombocytopenic purpura cases to be 64.54% and 83%, respectively. Investigation of antigen expression in the myeloid precursors of myelodysplastic syndrome patients showed the mean proportions of: 19.89%, 59.53%, 57.26%, 69.24%, 60.64% and 23.43% for CD117, CD34, HLA-DR, CD33, CD13 and CD11b, respectively. Also, idiopathic thrombocytopenic purpura cases showed the mean percentages of 11.73%, 45.67%, 58.90%, 74.28%, 70.16% and 15.66% for CD117, CD34, HLA-DR, CD33, CD13 and CD11b, respectively. Conclusion: There is no doubt that providing the reference values for an antigen expression pattern among myelodysplastic syndrome cases enhances the utility of flow cytometric analysis interpretation among these patients.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: To report a patient who was referred for orbital cellulitis but was finally diagnosed with acute leukemia. A 17-year-old boy presented with fever, periorbital erythema and swelling mimicking periorbital cellulitis. He underwent empiric antibiotic therapy. Complete blood counts revealed leukocytosis with a predominance of immature blast cells. Bone marrow aspiration confirmed the diagnosis of acute myelogenous leukemia. Chemotherapy was initiated resulting in resolution of signs and symptoms. Acute leukemia may mimic periorbital cellulitis and must be considered in the differential diagnosis.
    10/2013; 8(4):380-2.


Available from