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Myelodysplastic syndromes. Achievements in understanding and treatment . Myelodysplastic syndromes. Clonal hematopoietic stem cell disorder ch a racterized by ineffective hematopoiesis and peripheral cytopenias

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myelodysplastic syndromes

Myelodysplastic syndromes

Achievements in understanding and treatment

myelodysplastic syndromes2
Myelodysplastic syndromes
  • Clonal hematopoietic stem cell disorder characterized by ineffective hematopoiesis and peripheral cytopenias
  • Although a substantial proportion of MDS cases evolve to acute myeloid leukemia (AML), the natural history of these syndromes ranges from more indolent forms of the disease spanning years to those with a rapid evolution to AML

the leukemic disorder in which neoplastic clone that has been established may or may not fully progress to acute leukemia

myelodysplastic syndromes3

FAB classification system

Myelodysplastic syndromes
  • Refractory anemia (RA):cytopenia of one PB lineage; normo- or hypercellular marrow with dysplasias; < 1% PB blasts and <5% BM blasts
  • Refractory anemia with ringed sideroblasts (RARS): cytopenia, dysplasia and the same % blasts involvement in BM and PB as RA. Ringed sideroblasts account for > 15% of nucleated cells in marrow.
  • Refractory anemia with excess of blasts (REAEB): Cytopenia or two or more PB lineages; dysplasia involving all 3 lineages; < 5% PB blasts and 5-20% BM blasts
  • Refractory anemia with excess blasts in transformation: (REAEB-t): hematologic features identical to RAEB. >5% blasts in PB or 21-30% blasts in BM, or the presence of Auer rods in the blasts
  • Chronic myelomonocytic leukemia (CMML):monocytosis in PB>109/L; < 5% blast in PB and up to 20% BM balsts
myelodysplastic syndromes4

WHO classification system

Myelodysplastic syndromes

Myelodysplastic syndromes:

  • Refractory anemia (RA)

With ringed sideroblasts (RARS)

Without ringed sideroblasts

  • Refractory cytopenia (MDS) with multilineage dysplasia (RCMD)
  • Refractory anemia with excess blasts (RAEB)
  • 5q- syndrome

Myelodysplastic syndrome, unclassifiable

  • Myelodysplastic/Myeloprolipherative diseases
  • Chronic myelomonocytic leukemia (CMML)
  • Atypic chronic myelogenous leukemia (aCML)
myelodysplastic syndromes5

IPSS risk-based classification system

Myelodysplastic syndromes

Marrow blast percentage:

  • < 5 0
  • 5-10 0.5
  • 11-20 1.5
  • 21-30 2.0

Cytogentic fetures

  • Good prognosis 0

(–Y, 5q- , 20q-)

  • Intermediate prognosis 0.5

(+8, miscellaneous singleabnormality, double abnormalities)

  • Poor prognosis 1.0

(abnor. 7, complex- >3 abnor.)

Cytopenias

  • None or one type 0
  • 2 or 3 type 0.5
myelodysplastic syndromes6

Overall IPSS score and survival

Myelodysplastic syndromes

Overall score: Median survival:

low

  • 0 5.7 years

Intermediate

  • 1 (0.5 or 1) 3.5 years
  • 2 (1.5 or 2) 1.2 years

High

  • > 2.5 0.4 years
diagnosis of mds
Diagnosis of MDS
  • Aplastic anaemia and some disease accompanied by marrow dysplasia, including wit. B12 and/or folate deficiency, exposure to haevy metals, recent cytotoxic therapy and ongoing inflamation (including HIV and chronic liver disease/alcohol use) should be ruled out
mds clinical findings
MDS – clinical findings
  • These are non-specific, and are usually the consequences of cytopenias, including:
  • symptoms of anaemia
  • infections due to neutropenia, but also to the frequently associated defect in neutrophil function
  • bleeding due to thrombocytopenia (may also occur in moderately thrombocytopenic patients or even in patients with normal platelets count, because of thrombocytopathy)
bone marrow biopsy
Bone marrow biopsy
  • Blood examination and bone marrow aspirate are sufficient for a diagnosis of MDS
  • It is obviously important in cases of difficult diagnosis , and it could brink additional prognostic information in some cases
  • normal or increased cellularity is seen in 85-90% od cases
  • abnormal localization of immature precusors (ALIP)
  • Fibrosis (significant in 15-20% of cases)
dysplasia apoptosis and cytokines in mds
Dysplasia, apoptosis and cytokines in MDS
  • Despite increased proliferation of the marrow, there is an increased rate of prgrammed cell deathkinetically the apoptosis prevails over the increased proliferation, causing the peripheral cytopenia
  • Cytokines derived from unselected marrow mononuclear cells are belived to be extrinsic factors predisposing to apoptosis (TNF - inhibit normal and MDS colony growth; INF, IL1, TGF - have also be implicated in causing apoptosis)
evidence for an immune mediated suppression of the marrow in mds
Evidence for an immune – mediated suppression of the marrow in MDS
  • T cells inhibit MDS CFU-E
  • CD8+ cells inhibit CFU-GM
  • Immunosuppressive agents improve cytopenia in MDSand eliminate autosuppressive T cells
  • T cells are activated in MDS
  • T cell are show a skewed T cell receptor V- repertoire
  • HLA-DR 15 over representation in MDS and aplastic anemia