Myelodysplastic syndrome

Question 1

Blast accumulation in BM (<20% in BM) ~ AML pre-cursor
BM suppression - cytopenia
Unique abnormal cells (dysplasia)

Answer 1

Myelodysplastic syndrome

MDS is basically an accumulation of blast cells but not quite up to what AML is. The blast cells that accumulate look strange and will suppress BM production

Question 2

Pelger-Huet cells
Micro megakaryocytes
Ringed sideroblasts

Answer 2

Myelodysplastic syndrome

Question 3

Neutrophils MDS

Answer 3

Pelger-Huet hypolobulated nuclei

Lack of granules

Question 4

Platelets MDS

Answer 4

Micro-megakaryocytes

Question 5

RBCs MDS

Answer 5

Conjoined]
Irregular cytoplasm distribution
Ringed sideroblasts with iron around nucleus

Question 6

Blasts >20% vs. blasts <20%

Answer 6

AML vs. MDS

Question 7

8 types of MDS

Answer 7

Refractory anaemia (just RBCs affected)
Refractory anaemia with ringed sideroblasts (RBCs a bit more abnormal)
Refractory cytopenia with multilineage dysplasia (2 or more cell lines affected)
Refractory cytopenia with multilineage dysplasia + ringed sideroblasts (as above with >15% ringed sideroblasts)
RAEB I (cytopenia and 5-10% blasts in BM)
RAEB II (cytopenia and 10-20% blasts in BM)
5q syndrome (hyperplasia of hypolobulated micromegakaryocytes - platelets only)
Unclassified

Question 8

Sideroblasts in alcoholic

Answer 8

Secondary sideroblastic anaemia

Question 9

Anaemia

Erythroid dysplasia in RBCs

Answer 9

Refractory anaemia

Question 10

Anaemia

>15% RBCs are ringed sideroblasts in RBCs

Answer 10

Refractory anaemia with ringed sideroblasts

Question 11

Cytopenia and dysplasia in 2 or more cell lines

Answer 11

Refractory cytopenia with multilineage dysplasia

Question 12

Cytopenia and dysplasia in 2 or more cell lines

PLUS >15% ringed sideroblasts

Answer 12

Refractory cytopenia with multilineage dysplasia + ringed sideroblasts

Question 13

Cytopenia(s)

5-10% blasts in BM

Answer 13

RAEB I

Question 14

Cytopenia(s)

10-20% blasts in BM or Auer rods

Answer 14

RAEB II

Question 15

Megakaryocytes with hypolobulated nuclei

Answer 15

5q syndrome

Question 16

Complex

Answer 16

Unclassified

Question 17

Rule of 1/3s MDS

Answer 17

1/3 die from infection
1/3 die from bleeding (thrombocytopenia)
1/3 die from AML (>20% blasts)

Question 18

Rx that prolong survival

Answer 18

Intensive chemo therapy (kill proliferating cells)
Allogenic SCT (replace cells)

~ But most pts elderly so no eligible - very intense

Question 19

Ladder of MDS treatment

Answer 19

1. Supportive therapy – blood products (helps anaemia + thrombocytopenia), anti-microbial therapy (helps neutropenia), growth factors (incl. EPO + G-CSF to promote RBC/platelet/neutrophil production)
2. Biological modifiers – help symptoms – immunosuppressive agents, hypo-methylating agents (like ‘gentle chemo’ e.g. azacytidine, decitabine), lenalidomide
3. Oral chemo (e.g. hydroxyurea)
4. Low dose chemo (e.g. s/c cytarabine)
5. SCT + intensive chemo if fit enough + bad prognosis MDS