1. Myelodysplastic Syndrome (MDS)
Dr. Fatma Al-Qhtani

2. MDS Disorders of multipotent hemopoietic stem cell Heterogenous group
Hemopoiesis is ineffective and morphological dysplastic ( Blood/ BM)
Tendency to evolve into acute leukemia
Elderly patient

3. FAB (1982) Refractory anemia (RA)
Refractory anemia with ring sideroblast (RARS)
Refractory anemia with excess blast (RAEB)
Refractory anemia with excess blast in transformation (RAEBt)
Chronic myelomonocytic leukemia (CMML)

4. Refractory Anemia Peripheral blood: Anemia Blast< 1%
Monocytes < 1 X 109
Bone Marrow:
Blast < 5%
Ringed sideroblasts < 15% of erythroblast

5. RARS Peripheral blood: Anemia Blast < I% Monocytes < 1x 109
Bone marrow:
Blast < 5%
Ringed sideroblasts > 15% of erythroblast

6. RAEB Peripheral blood: Anemia Monocytes <1 x 10 9
Blasts > 1% , but < 5%
Bone marrow:
Blasts > 5% and/ or < 20%

7. CMML Peripheral blood: Monocytes > 1 x109 Blast < 5 %
Bone marrow:
Blast up to 20%
Increased of promonocytes

8. WHO Refractory anemia Refractory anemia e ringed siderblast
Refractory cytopenia e multilineage dysplasia
Refractory cytopenia e multilineage dysplasia & ringed sideroblasts
Refractory anemia e excess blast-1
Refractory anemia e excess blast-2
Myelodysplastic syndrome unclassified
MDS associated e isolated del (5q)

9. WHO Bone Marrow Blood Subtype Anemia RA RARS RCMD RCMD-RS
Erythroid dysplasia only
Anemia RA
Erythroid dys
>15% ringed
RARS
>10%Dysp in 2 or more cell lineage
Bi- pancytopenia
RCMD
>10%Dys 2 or more cell lineage
Bi-pancytopenia
RCMD-RS

10. WHO Bone Marrow Blood subtype RAEB-1 RAEB-2 MDS-U MDS with 5q
Uni-multilineage dys, 5-9%blast
Cytopenia
<5% blast
RAEB-1
Uni-multi dys
10-19%blast
Or Auer rods
Cytopenia,
5-19%blast or Auer rods
RAEB-2
Myeloid or megakaryocte dys
cytopenia
MDS-U
Mega e hypolobated nuclei, <5%blast
Anemia,nor or increased PLT
MDS with 5q

11. Relation FAB & WHO WHO FAB RA RARS RAEB RAEBt CMML RA(unilineage) RCMD
5q-syndrome RA
RARS(unilineage)
RCMD-RS
RARS
RAEB-1
RAEB-2
RAEB
AML e multilieage dys
AML & MDS-TR
RAEBt
Myelodysplastic/ myeloproliferative disease
CMML

12. Etiology Primary  unknown Secondary or therapy related MDS
Case control study shown increased incidence in: smoker, agricultural workers, plant and machine operator, ionizing radiation, organic chemical
Secondary or therapy related MDS
Cytotoxic chemotherapy
Median time is 4-5 years

13. Clinical feature Incidental blood count
Bone marrow failure symptoms & signs
Fatigue due to anemia
Bleeding
infections

14. CMML Splenomegaly (10%) Maculopapular skin infiltration
Monocytic pleural or pericardial effusion
JMML (MPD/MDS)
Pallor, bleeding, hepatosplenomegaly, skin involvement

15. Laboratory Feature Anemia 30- 50 % are pancytopenic
20% anemia in combination e neutropenia or thrombocytopenia
< 5% isolated neutropenia or thrombocytopenia

16. Erythroid Oval macrocytosis Anisopoikiloctosis
Hypochromic red cell fragments
Basophilic stippling
NRBCs

17. Granulocytic Nuclear hypolobulation (Pelger) Nuclear Hypersegmentation
Ring nuclei coarse chromatin clumping
Cytoplasmic hypogranulation or agranulition

18. Monocytic
Abnormal nuclear lobulation

19. Megakaryocte
Agranular platelets
Giant platelets

20. Erythroid (BM) Ringed sideroblast Vacuolated cytoplasm
Multinuclearity ( bi or tri)
Internuclear bridging
Erythroid hypoplasia

21. Myeloid (BM) Loss of primary & secondary granules increased Blasts
Increased eosinophils & or Basophils

22. Megakaryocytes (BM) Micromegakaryocytes
Large mono or binuclear megakaryocytes
Large megakaryocytes with widely dispersed nuclei

23. Prognosis Poor:( FBC & BF) Anemia Neutropenia Thrombocytopenia
Presence of blast
Raised lactic dehydrogenase

24. Prognosis Poor ( Marrow Aspirate): Blast > 10% Trilineage dysplasia
Trephine
Abnormal localization of immature precursors ( Erythroid/ megakaryocytes)

25. Prognosis Chromosome analysis: Monosomy 7 Complex Karyotype
Karyotypic evolution
Molecular Studies:
N- RAS mutation
P53 mutation

26. Management Observation Supportive care Intensive chemotherapy
Allogenic or autologous transplant
Cytotoxic therapy
Immunosupperessive agents

27. THANK YOU