MDS

1. MYELODYSPLASTIC SYNDROME
Dr.T.Arivazhagan
Department of Pathology

3. Definition
Clonal hematopoietic stem cell disorders characterized by
cytopenias, dysplasia in one or more of the major myeloid
cell lines, ineffective hematopoiesis with cellular marrow and
risk for leukemic transformation.

4. 1. Bone marrow cannot produce blood cells effectively
2. If produced the blood cells will be defective in nature
3. These abnormal cells usually destroyed before they leave
from bone marrow or shortly after entering into the blood
stream

5. 1. Varying degree of tri lineage cytopenias
2. Dysplasia
3. Normocellular or Hypercellular Bone marrow
4. May progress to acute leukemia's

6. Incidence
1. Disease of elderly
2. Median age is 65 years
3. M>F
4. Incidence 1/1000/years > 60 years

7. PREDISPOSING FACTORS
HEREDITARY
1. Down’s syndrome
2. NF
3. Congenital Neutropenia
4. Fanconi’s anemia
5. Shwachman Diamond syndrome
6. Diamond Blackfan syndrome
ACQUIRED
1. Radiation
2. Mutation
3. Alkylating agents
4. Tabacoo
5. Benzene
6. Aplastic anemia
7. PNH

8. CLASSIFICATION
1. FAB
2. WHO

9. FAB CLASSIFIACTION
SUB TYPE PS BM
RA Blasts < 1%
Blasts < 5%
RS < 15%
RA – RS Blasts < 1%
Blasts < 5%
RS > 15%
RAEB Blasts < 5% Blasts 5-20%
RAEBT Blasts > 5%
Blasts 20-30%
Auer rods
CMML
Blasts < 5%
Monocyte > 1%
Any of the above
AML Variable Blasts > 30%

10. WHO CLASSIFICATION
SUBTYPE PS BM
RCUD Unicytopenia / No blast
Unilineage dysplasia > 10% of cells
Blasts < 5%
RS < 15%
RARS Anemia
Erythroid hyperplasia with
Dyserythropoiesis
RS > 15%
Blasts < 5%
RCMD
Bi / Pancytopenia
No blast / No Auer rods
Dysplasia >10 % in > 2 lineage
Blast <5%
RS > 15%
RAEB-1
<5% blasts
Bi / Pancytopenia
No Auer Rods
5-9% blasts
No Auer rods
Dysplasia > 1 hematopoietic cell line
RAEB-2
5-9% blasts
Cytopenias
Auer rods
10-19% blasts
Auer rod +
Myelodysplasia > 1 line

11. MDS – U
No blasts
Cytopenias only
< 5% blasts
Cytogenetic abnormalities
Granulocytic or
Megakaryocytic dysplasia
MDS – 5q deletion
< 1% blasts
Anemia
Increased platelets
< 5% blasts
Isolated 5q deletion
No Auer rods
CMDS
< 2 % blasts
Dysplastic changes in > 10 %
of neutrophils
Dysplasia > 10% erythroid
precursors
Dysplasia > 10% granulocytic
precursors

12. Etio pathogenesis
1. Denovo
2. Therapy related

13. Characteristic features
1. Dyserythropoiesis
2. Dysmyelopoiesis
3. Dysmegakaryopoiesis
4. Ring sideroblast
5. Type I & II blast

14. PERIPHERAL SMEAR
Erythroid
• Ovalocytosis
• Macrocytosis
• Eliptocytosis
• Tear drop cells
• Nucleated red cells
• Basophilic stippling
• Howell –Jolly bodies
Myeloid
• Pseudo – Pelger
Huet anomaly
• Auer rod
• Hypogranulation
• Hypersegmentation
of nuclei
Megakaryocyte
• Giant platelets
• Hypogranular
platelets
• Agranullar platelets

15. Bone marrow
Erythroid
• Megaloblastic
erythropoiesis
• Nuclear budding
• Ring sideroblast
• Internuclear bridging
• Nuclear fragments
• Multinucleation
Myeloid
• Defective granulation
• Hypolobation
• Auer rods
• Maturation arrest
• ALIP
Megakaryocyte
• Micromegakaryocyte
• Hypogranulation
• Hypolobation
• Multiple small nuclei

19. Clinical presentation
1. Anemia
2. Lethargy
3. Weakness
4. Fainting
5. Palpitation
6. Dizziness
7. Headache
8. Menstrual abnormalities
9. Poor appetite
10.Irritability

23. Treatment
1. Supportive therapy
2. Chemotherapy
3. Bone marrow transplantation