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Myelodysplastic syndrome (MDS) (TREATMENT (Supportive: <5% blasts …
Myelodysplastic syndrome (MDS)
a group of acquired bone marrow disorders that are due to a defect in stem cells. characterized by increasing bone marrow failure with quantitative and qualitative abnormalities of all three myeloid cell lines (red cells, granulocyte/monocytes and platelets).
single-lineage cytopenias, bicytopenia or pancytopenia
incresed risk for developing AML 30% of cases.
mainly in the elderly
Pathogenesis and Etiology
Genetic mutations and their accumulation
mutaions affecting mRNA splicing
Accelerated apoptosis AND increased angiogenesis
A poor survival in those having mutations in TP53, E2H2, ETV6, RUNX1 and ASXL1.
Primary MDS: familial defects (impaired hematopoiesis or DNA repair), age ( 60-75), environmental factors ( tobacco, metal)
Secondary MDS (therapy-related):
chemotherapy : alkylating agents, topoisomerase inhibitors
ionizing radiation
Clinical Features
Anemia (Hb<10%,MCV N/>) 80%
Neutropenia 40%
Thrombocytopenia 30-45%
Asymptomatic
Symptoms suggestive of a blood disorder (fatigue, exercise intolerance, infections, inappropriate bleeding or bruising)
Bone marrow examination
Aspiration-cytological assessment:
dyserytropoiesis (megaloblastic maturation, abnormalities of erythroblast nuclei, iron-laden mitochondria=sideroblasts)
dysgranulopoiesis (hypogranulation, hypo- or hypersegmentation, excess blasts)
dysmegakariopoiesis (giant or small, hypogranular)
Trephine core BM biopsy: mostly normo- or hypercellular, sometimes hypocellular
ALIP (abnormal localization of immature precursors)
CLASSIFICATION (WHO) *
TREATMENT
Supportive: <5% blasts
transfusion support (blood, platelets)
iron chelatation (Desferrioxamine)
tranexamic acid
growth factors (G-CSF, EPO)
immunosuppressive (ATG), immunomodulating (Lenalidomide (a thalidomide analogue)) , chemotherapy.
allogenic hematopoietic cell transplantation (alloHCT)
BM transplantation