myelodysplastic syndrome (mds) dr. fatma al-qhtani
TRANSCRIPT
![Page 1: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/1.jpg)
Myelodysplastic Syndrome (MDS)
Dr. Fatma Al-Qhtani
![Page 2: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/2.jpg)
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
![Page 3: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/3.jpg)
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)
![Page 4: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/4.jpg)
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
![Page 5: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/5.jpg)
RARS Peripheral blood:
1. Anemia
2. Blast < I%3. Monocytes < 1x 109
Bone marrow:
1. Blast < 5%
2. Ringed sideroblasts > 15% of erythroblast
![Page 6: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/6.jpg)
RAEB Peripheral blood:
1. Anemia2. Monocytes <1 x 10 9
3. Blasts > 1% , but < 5% Bone marrow:
1. Blasts > 5% and/ or < 20%
![Page 7: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/7.jpg)
CMML Peripheral blood:1. Monocytes > 1 x109
2. Blast < 5 % Bone marrow:
1. Blast up to 20%
2. Increased of promonocytes
![Page 8: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/8.jpg)
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)
![Page 9: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/9.jpg)
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
![Page 10: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/10.jpg)
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
![Page 11: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/11.jpg)
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
![Page 12: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/12.jpg)
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
![Page 13: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/13.jpg)
Clinical feature
1. Incidental blood count
2. Bone marrow failure symptoms & signs
3. Fatigue due to anemia
4. Bleeding
5. infections
![Page 14: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/14.jpg)
CMML Splenomegaly (10%) Maculopapular skin infiltration Monocytic pleural or pericardial
effusion JMML (MPD/MDS)1. Pallor, bleeding,
hepatosplenomegaly, skin involvement
![Page 15: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/15.jpg)
Laboratory Feature Anemia 30- 50 % are pancytopenic 20% anemia in combination e
neutropenia or thrombocytopenia < 5% isolated neutropenia or
thrombocytopenia
![Page 16: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/16.jpg)
Erythroid Oval macrocytosis Anisopoikiloctosis Hypochromic red cell fragments Basophilic stippling NRBCs
![Page 17: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/17.jpg)
Granulocytic Nuclear hypolobulation (Pelger) Nuclear Hypersegmentation Ring nuclei coarse chromatin clumping Cytoplasmic hypogranulation or
agranulition
![Page 18: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/18.jpg)
Monocytic Abnormal nuclear lobulation
![Page 19: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/19.jpg)
Megakaryocte Agranular platelets Giant platelets
![Page 20: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/20.jpg)
Erythroid (BM) Ringed sideroblast Vacuolated cytoplasm Multinuclearity ( bi or tri) Internuclear bridging Erythroid hypoplasia
![Page 21: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/21.jpg)
Myeloid (BM) Loss of primary & secondary granules increased Blasts Increased eosinophils & or Basophils
![Page 22: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/22.jpg)
Megakaryocytes (BM) Micromegakaryocytes Large mono or binuclear
megakaryocytes Large megakaryocytes with widely
dispersed nuclei
![Page 23: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/23.jpg)
Prognosis Poor:( FBC & BF)
1. Anemia
2. Neutropenia
3. Thrombocytopenia
4. Presence of blast
5. Raised lactic dehydrogenase
![Page 24: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/24.jpg)
Prognosis Poor ( Marrow Aspirate):
1. Blast > 10%
2. Trilineage dysplasia Trephine
1. Abnormal localization of immature precursors ( Erythroid/ megakaryocytes)
![Page 25: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/25.jpg)
Prognosis Chromosome analysis:
1. Monosomy 7
2. Complex Karyotype
3. Karyotypic evolution Molecular Studies:
1. N- RAS mutation
2. P53 mutation
![Page 26: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/26.jpg)
Management
1. Observation
2. Supportive care
3. Intensive chemotherapy
4. Allogenic or autologous transplant
5. Cytotoxic therapy
6. Immunosupperessive agents
![Page 27: Myelodysplastic Syndrome (MDS) Dr. Fatma Al-Qhtani](https://reader035.vdocument.in/reader035/viewer/2022062408/56649f2f5503460f94c49198/html5/thumbnails/27.jpg)
THANK YOU