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Myelodysplastic Syndrome (MDS)
Dr. Fatma Al-Qhtani
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
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)
Refractory Anemia Peripheral blood:
1. Anemia
2. Blast< 1%3. Monocytes < 1 X 109
Bone Marrow:
1. Blast < 5%
2. Ringed sideroblasts < 15% of erythroblast
RARS Peripheral blood:
1. Anemia
2. Blast < I%3. Monocytes < 1x 109
Bone marrow:
1. Blast < 5%
2. Ringed sideroblasts > 15% of erythroblast
RAEB Peripheral blood:
1. Anemia2. Monocytes <1 x 10 9
3. Blasts > 1% , but < 5% Bone marrow:
1. Blasts > 5% and/ or < 20%
CMML Peripheral blood:1. Monocytes > 1 x109
2. Blast < 5 % Bone marrow:
1. Blast up to 20%
2. Increased of promonocytes
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)
WHOSubtype Blood Bone Marrow
RA Anemia Erythroid dysplasia only
RARS Anemia Erythroid dys
>15% ringed
RCMD Bi- pancytopenia >10%Dysp in 2 or more cell lineage
RCMD-RS Bi-pancytopenia >10%Dys 2 or more cell lineage
>15% ringed
WHOsubtype Blood Bone Marrow
RAEB-1 Cytopenia
<5% blast
Uni-multilineage dys, 5-9%blast
RAEB-2 Cytopenia,
5-19%blast or Auer rods
Uni-multi dys
10-19%blast
Or Auer rods
MDS-U cytopenia Myeloid or megakaryocte dys
MDS with 5q Anemia,nor or increased PLT
Mega e hypolobated nuclei, <5%blast
Relation FAB & WHOFAB WHO
RA RA(unilineage)
RCMD
5q-syndrome
RARS RARS(unilineage)
RCMD-RS
RAEB RAEB-1
RAEB-2
RAEBt AML e multilieage dys
AML & MDS-TR
CMML Myelodysplastic/ myeloproliferative disease
Etiology Primary unknown1. Case control study shown increased
incidence in: smoker, agricultural workers, plant and machine operator, ionizing radiation, organic chemical
Secondary or therapy related MDS1. Cytotoxic chemotherapy
2. Median time is 4-5 years
Clinical feature
1. Incidental blood count
2. Bone marrow failure symptoms & signs
3. Fatigue due to anemia
4. Bleeding
5. infections
CMML Splenomegaly (10%) Maculopapular skin infiltration Monocytic pleural or pericardial
effusion JMML (MPD/MDS)1. Pallor, bleeding,
hepatosplenomegaly, skin involvement
Laboratory Feature Anemia 30- 50 % are pancytopenic 20% anemia in combination e
neutropenia or thrombocytopenia < 5% isolated neutropenia or
thrombocytopenia
Erythroid Oval macrocytosis Anisopoikiloctosis Hypochromic red cell fragments Basophilic stippling NRBCs
Granulocytic Nuclear hypolobulation (Pelger) Nuclear Hypersegmentation Ring nuclei coarse chromatin clumping Cytoplasmic hypogranulation or
agranulition
Monocytic Abnormal nuclear lobulation
Megakaryocte Agranular platelets Giant platelets
Erythroid (BM) Ringed sideroblast Vacuolated cytoplasm Multinuclearity ( bi or tri) Internuclear bridging Erythroid hypoplasia
Myeloid (BM) Loss of primary & secondary granules increased Blasts Increased eosinophils & or Basophils
Megakaryocytes (BM) Micromegakaryocytes Large mono or binuclear
megakaryocytes Large megakaryocytes with widely
dispersed nuclei
Prognosis Poor:( FBC & BF)
1. Anemia
2. Neutropenia
3. Thrombocytopenia
4. Presence of blast
5. Raised lactic dehydrogenase
Prognosis Poor ( Marrow Aspirate):
1. Blast > 10%
2. Trilineage dysplasia Trephine
1. Abnormal localization of immature precursors ( Erythroid/ megakaryocytes)
Prognosis Chromosome analysis:
1. Monosomy 7
2. Complex Karyotype
3. Karyotypic evolution Molecular Studies:
1. N- RAS mutation
2. P53 mutation
Management
1. Observation
2. Supportive care
3. Intensive chemotherapy
4. Allogenic or autologous transplant
5. Cytotoxic therapy
6. Immunosupperessive agents
THANK YOU
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