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38 Cards in this Set
- Front
- Back
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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 |
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Myelodysplastic features
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Blood cytopenia
marrow hyperplasia no lymphadenopathy or splenomegaly |
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Myelodysplastic syndromes progress to?
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AML, very bad prognosis
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Causes of Myelodysplasia
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-Primary (idiopathic)
-Secondary to drug, radiation, heavy metals, viral infections, megaloblastic anemias, congenital dyserythropoietic anemia, PNH |
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Mean age onset of myelodysplastic syndromes
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About 70 years old
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Dyserythropoeisis
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Abnormal RBC prodruction
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Features of myelodysplasia
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-dyserythropoeisis
-irregular nuclear borders -multinucleation -megaloblastoid change (inc n/c ratio) -ringed sideroblasts -vacualization -PAS + |
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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 |
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Dysgranulopoeisis morph features
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-small cell size, hypogranulation, nuclear hypersegmentation or hypolabation, pseudo Cheidiak-Higashi granules, Pseudo Pelger-Huet cells
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Dysmegakaryocytopoiesis morph features
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Hypolobation or multinucleation, hypogranular platelets
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ALIP
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Atypical localization of immature precursors
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ALIP is...
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clusters of myeolblasts and promyelocytes away from vascular structures
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ALIP is commonly seen in?
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-Higher grade Myelodysplastic syndromes
-indicates a more rapid evolution to acute leukemia |
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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 |
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Risks of high grade myelodysplastic syndromes
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Transformation to AML, which is refractory to treatment
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Natural progression of MDS without treatment
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Low grade MDS evolves to High grade MDS which evolves to acute leukemia (AML) refractory to treatment
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Refractory anemia
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-Chronic anemia due to ineffective erythropoeisis (normocytic or macrocytic) which is unresponsive to Iron, Vit B12, folate
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Dysplasia in Refractory anemia
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Only dyserythropoeisis
<1% in pb <5% in BM |
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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% |
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Refractory anemia with ringed sideroblasts
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Like refractory anemia but 15% or more of the erythroid precursors in the BM are ringed sideroblasts
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Secondary causes of ringed sideroblasts (must be excluded for refractory anemia with ringed sideroblast diagnosis)
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Anti-TB drugs and Alcoholism
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Dysplasia seen in Refractory anemia with ringed sideroblasts
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-Dysplasia limited to dyserythropoiesis
-Myeloblasts <1% in PB, <5% in BM |
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Refractory anemia with Ringed sideroblasts cytogenics and survival
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-<10% have cytogenetics
Survival ~6yrs 1-2% progression to acute leukemia |
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Refractory cytopenia with multilineage dysplasia
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Bi- or Pancytopenia with dysplastic changes in TWO or THREE lineages
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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 |
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Refractory anemia with excess blasts
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dysplasia in all three lineages
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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 |
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RAEB-2
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AUER RODS or myeloblasts <5-19% in PB +/- 10-19% in BM or AUER RODS
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Auer rods
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Abnormally packaged myeloperoxidase crystals
Looks like needle shaped red rods in blast cytoplasm |
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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 |
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Myelodysplastic/myeloproliferative overlap syndromes
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Diseases with myelodysplastic features (abnormal dysplastic morphologic forms) but also myeloproliferative features (usually leukocytosis +/- splenomegaly)
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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 |
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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, |
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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 |
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CML-1
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Myeloblasts <5% in blood
<10% in BM |
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CML-2
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Myeloblasts <5-19% in blood, 10-19% in marrow
or AUER RODS |
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CML cytogenics and median Progression
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20-40% abnormalities
15-30%progress to AML |
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Auer rods in blood
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Auer rods = AML EXCEPT
RAEB-2 or CMML-2 |
