Refractory cytopenia with unilineage dysplasia. Refractory cytopenia with multilineage dysplasia. Myelodysplastic Syndromes: Comparison of the FAB and WHO Classifications FAB (1982)ĪML = acute myeloid leukemia FAB = French-American-British classification scheme MDS = myelodysplastic syndromes WHO = World Health Organization. The World Health Organization (WHO) classification has supplanted the historic French-American-British (FAB) classification, as shown in Table 1. The clinical classification of the MDS depends on whether there is an identifiable etiology and whether the MDS has been treated previously. The morphological classification of MDS is largely based on the percent of myeloblasts in the bone marrow and blood, the type and degree of myeloid dysplasia, and the presence of ring sideroblasts. Myelodysplastic syndromes (MDS) are classified according to features of cellular morphology, etiology, and clinical presentation.
Refractory anemia with ring sideroblasts (RARS).Pathological and Prognostic Systems for MDS Strom SS, Gu Y, Gruschkus SK, et al.: Risk factors of myelodysplastic syndromes: a case-control study.Du Y, Fryzek J, Sekeres MA, et al.: Smoking and alcohol intake as risk factors for myelodysplastic syndromes (MDS).Nand S, Godwin JE: Hypoplastic myelodysplastic syndrome.Tiu RV, Gondek LP, O'Keefe CL, et al.: Prognostic impact of SNP array karyotyping in myelodysplastic syndromes and related myeloid malignancies.
Gyger M, Infante-Rivard C, D'Angelo G, et al.: Prognostic value of clonal chromosomal abnormalities in patients with primary myelodysplastic syndromes.Tuncer MA, Pagliuca A, Hicsonmez G, et al.: Primary myelodysplastic syndrome in children: the clinical experience in 33 cases.Sekeres MA, Schoonen WM, Kantarjian H, et al.: Characteristics of US patients with myelodysplastic syndromes: results of six cross-sectional physician surveys.Ma X, Does M, Raza A, et al.: Myelodysplastic syndromes: incidence and survival in the United States.Organic chemicals (e.g., benzene, toluene, xylene, and chloramphenicol).Potential environmental risk factors for developing MDS include exposure to the following: Profound cytopenias and may respond more frequently to immunosuppressive therapy.Īpproximately 90% of MDS cases occur de novo with no identifiable cause. Hypoplastic myelodysplastic patients tend to have Usually hypercellular at diagnosis, 10% of patients present with a Single-nucleotide polymorphism array technology may increase the detection of genetic abnormalities to 80%. Or part of chromosome 5 or 7, or trisomy 8. Approximately 50% of patients have a detectable cytogenetic abnormality, most commonly a deletion of all Hepatosplenomegaly may indicate an overlapping myeloproliferative neoplasm. Anemia, bleeding,Įasy bruising, and fatigue are common initial findings. With a median age at diagnosis of approximately 70 years, although patients as young as 2 years have been reported. MDS occur predominantly in older patients (usually older than 60 years),
(micromegakaryocytes) may be seen in the marrow, and hypogranular or giant Early, abnormal myeloid progenitorsĪre identified in the marrow in varying percentages. Circulating granulocytesĪre often hypogranular or hypergranular and may display theĪcquired pseudo-Pelger-Huët abnormality. Normal vitamin B 12 and folate levels, is frequently observed. Megaloblastoid erythroid hyperplasia with macrocytic anemia, associated with MDS are characterized by abnormal bone marrow and blood cell morphology. The acute leukemic phase is less responsive toĬhemotherapy than is de novo AML. For more information, see the Pathological and Prognostic Systems for MDS section. Many patients succumb to complications of cytopenias before progression to this stage. By convention, MDS are reclassified as acute myeloid leukemia (AML) with myelodysplastic features when blood or bone marrow blasts reach or exceed 20%. Related to the number of bone marrow blast cells, to certain cytogenetic abnormalities, and to the amount of Syndromes may arise de novo or secondarily after treatment with chemotherapyĪnd/or radiation therapy for other cancers or, rarely, after environmental exposures. They are more common in men and White individuals. MDS are diagnosed in slightly more than 10,000 people in the United States yearly, for an annual age-adjusted incidence rate of approximately 4.4 to 4.6 cases per 100,000 people. The MDS are a collection of myeloid malignancies characterized by one Go to Patient Version Incidence and Mortality
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