Myelodysplastic Syndromes

Question 1

Definition of Myelodysplastic disorders

Answer 1

Heteregenous ghroup of progressive disorders featuring ineffective proliferation and differentiation of abnormally maturing myeloid stem cells
Elderly
Week to months
20% is Acute leukaemia)

Question 2

Features of myelodysplastic disorders

Answer 2

Peripheral cytopenia, qualitative abnormalities of cell maturation
Risk of aml transformation
BM failure and cytopenias
Hypercellular BM
Defective cells - RBCs - ring sideroblasts
- WBCs - Hypogranulation, pseudo-pelger-huet
- Platelets - Micromegakaryocytes, hypolobated nuclei

Question 3

Blood: Anaemia, no blasts
BM: Erythroid dysplasia with

Answer 3

Refractory anaemia

Question 4

Blood: Anaemia, no blasts
BM: Erythroid dysplasia with >15% ringed sideroblasts

Answer 4

Refractory anaemia + ringed sideroblasts

Question 5

Blood: Cytopenia in >2 cell lines
BM: Dysplasia in >10% cells in >2 cell lines

Answer 5

Refractory cytopenia with multilineage dysplasia

Question 6

Blood: Cytopenia in >2 cell lines
BM: Dysplasia in >10% cells in >2 cell lines with >15% ringed sideroblasts

Answer 6

Refractory cytopenia with multilineage dysplasia + ringed sideroblasts

Question 7

Blood: Cytopenia

Answer 7

Refractory anaemia with excess blasts - 1

Question 8

Blood: Cytopenia 5-19% blasts or Auer rods
BM: Dysplasias, 10-19% Blasts or Auer rods

Answer 8

Refractory anaemia with excess blasts - 2

Question 9

Blood: Anaemia, normal or increased platelets
BM: Megakaryocytes with hypolobated nuclei and

Answer 9

MDS with 5q deletion

Question 10

Blood: Complex - cytopenias, no balsts/Auer rods
BM: Complex - myeloid or megakaryocytic dysplasia,

Answer 10

Myelodysplasia syndrome Unclassified

Question 11

Treatment of Myelodysplastic syndromes

Answer 11

Supportive - transfusions, EPO, G-CSF, ABx
Biological modifiers - immunosuppression, lenalidomide, azacytidine
Chemo - similar to AML
Allogenic SCT

Question 12

Prognosis of Myelodysplastic syndromes

Answer 12

International Prognostic Scoring System
BM Blast %, karyotype, cytopenia
1/3 die of infection
1/3 die of bleeding
1/3 die of acute leukaemia