Myelodisplastic Syndromes
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- Myelodysplastic Syndromes
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Clonal stem cell diseases with ineffective hematopoeisis
-abn hematopoeitic cells are ind the BM, don't migrate well to PB - Myelodysplastic features
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Blood cytopenia
marrow hyperplasia
no lymphadenopathy or splenomegaly - Myelodysplastic syndromes progress to?
- AML, very bad prognosis
- Causes of Myelodysplasia
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-Primary (idiopathic)
-Secondary to drug, radiation, heavy metals, viral infections, megaloblastic anemias, congenital dyserythropoietic anemia, PNH - Mean age onset of myelodysplastic syndromes
- About 70 years old
- Dyserythropoeisis
- Abnormal RBC prodruction
- Features of myelodysplasia
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-dyserythropoeisis
-irregular nuclear borders
-multinucleation
-megaloblastoid change (inc n/c ratio)
-ringed sideroblasts
-vacualization
-PAS + - How do you know the difference between primary and secondary myelodysplastic syndromes?
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-Cytogenetic abnormalaties point to primary MDS
-No cyto abnormalaties means it's primary or secondary MDS - Dysgranulopoeisis morph features
- -small cell size, hypogranulation, nuclear hypersegmentation or hypolabation, pseudo Cheidiak-Higashi granules, Pseudo Pelger-Huet cells
- Dysmegakaryocytopoiesis morph features
- Hypolobation or multinucleation, hypogranular platelets
- ALIP
- Atypical localization of immature precursors
- ALIP is...
- clusters of myeolblasts and promyelocytes away from vascular structures
- ALIP is commonly seen in?
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-Higher grade Myelodysplastic syndromes
-indicates a more rapid evolution to acute leukemia - Grades of Primary myelodysplastic syndromes
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Low grade- 1-refractory anemia 2-refractory anemia with ringed sideroblasts
High grade- 1-refractory cytopenia with multilineage dysplasia 2-refractory anemia with excess blasts - Risks of high grade myelodysplastic syndromes
- Transformation to AML, which is refractory to treatment
- Natural progression of MDS without treatment
- Low grade MDS evolves to High grade MDS which evolves to acute leukemia (AML) refractory to treatment
- Refractory anemia
- -Chronic anemia due to ineffective erythropoeisis (normocytic or macrocytic) which is unresponsive to Iron, Vit B12, folate
- Dysplasia in Refractory anemia
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Only dyserythropoeisis
<1% in pb
<5% in BM - Cytogenic abnormalities in refractory anemia
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-about 25% have them
-Median survival of 5-6 years
-Progression to acute leukemia in <10% - Refractory anemia with ringed sideroblasts
- Like refractory anemia but 15% or more of the erythroid precursors in the BM are ringed sideroblasts
- Secondary causes of ringed sideroblasts (must be excluded for refractory anemia with ringed sideroblast diagnosis)
- Anti-TB drugs and Alcoholism
- Dysplasia seen in Refractory anemia with ringed sideroblasts
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-Dysplasia limited to dyserythropoiesis
-Myeloblasts <1% in PB, <5% in BM - Refractory anemia with Ringed sideroblasts cytogenics and survival
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-<10% have cytogenetics
Survival ~6yrs
1-2% progression to acute leukemia - Refractory cytopenia with multilineage dysplasia
- Bi- or Pancytopenia with dysplastic changes in TWO or THREE lineages
- Refractory anemia with multilineage dysplasia cytogenics and survival
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Myeloblasts <1% PB, <5% BM,
ab half havecytogenetic abnormalaties
Survival ~3years-10%progress to acute leukemia - Refractory anemia with excess blasts
- dysplasia in all three lineages
- Types of Refractory anemia with excess blasts
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RAEB-1-myeloblasts <5% in PB +/- 5-9% in BM
RAEB-2-myeloblasts <5-19% in PB +/- 10-19% in BM or AUER RODS - RAEB-2
- AUER RODS or myeloblasts <5-19% in PB +/- 10-19% in BM or AUER RODS
- Auer rods
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Abnormally packaged myeloperoxidase crystals
Looks like needle shaped red rods in blast cytoplasm - RAEB cytogenics and survival
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~half with cytogenic abnormalaties
RAEB-1 median survival 18 months, 25% progress to AML
RAEB-2 median survival 10 months, 30-35% progress to AML - Myelodysplastic/myeloproliferative overlap syndromes
- Diseases with myelodysplastic features (abnormal dysplastic morphologic forms) but also myeloproliferative features (usually leukocytosis +/- splenomegaly)
- Overlap Syndromes classifications
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-Not applied to pts with history of myeloproliferative disorder (transformation) or philadelphia chromosome (CML)
May terminate with cytopenias or blast transformation - Chronic Myelomonocytic Leukemia
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Previous classified as MDS
-Persistant, non-reactive, peripheral MONOCYTE cont >1000mL
No philadelphia chromosome, <20% blasts in blood or BM, - Dysplasia seen in CML
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Dysplasia in one or more lineage
wbc count variable
splenomegaly common when WBC is high d/t leukemic infiltration - CML-1
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Myeloblasts <5% in blood
<10% in BM - CML-2
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Myeloblasts <5-19% in blood, 10-19% in marrow
or AUER RODS - CML cytogenics and median Progression
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20-40% abnormalities
15-30%progress to AML - Auer rods in blood
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Auer rods = AML EXCEPT
RAEB-2 or CMML-2