myelodysplastic syndromes – Flashcards

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question
what are myelodysplastic syndromes?
answer
a heterogenous collection of clonal hematopoietic stem cell malignant disorders marked by ineffective hematopoiesis leading to anemia, thrombocytopenia, and neutropenia. 33% go on to develop acute leukemia (generally AML), and they proceed marrow failure. these used to be called *refractory anemias.
question
what is the most common hematologic malignancy in the elderly?
answer
myelodysplastic syndrome - median age 60-75 *not rare - tired bone marrow, due radiotherapy etc
question
what is primary myelodysplasia?
answer
idiopatic, non-comples cytogenetics, low IPSS, and responds better to therapy
question
what is secondary myelodysplasia?
answer
usually due to chemo, has comples cytogenetics, high IPSS, and refractory to therapy
question
what defines CMML?
answer
peripherial blood monocytosis, presents with <5% blasts in peripheral blood and up to 20% blasts in the bone marrow
question
what are auer rods seen in?
answer
AML
question
what is refractory anemia?
answer
erythroid dysplasia, <5% blasts, <15% ringed sideroblasts, no auer rods
question
what is refractory anemia w/ringed sideroblasts?
answer
<5% blasts, <15% ringed sideroblasts, no auer rods
question
what is refractory cytopenia with multilineage displasia?
answer
dysplasia in >10% of cells in 2+ cell lines
question
what is refractory cytopenia with multilineage displasia w/ringed sideroblasts?
answer
dysplasia in >10% of cells in 2+ cell lines with >15% ringed sideroblasts
question
what is refractory anemia with excess blasts types 1 and 2?
answer
RAEB 1: 5-9% blasts, no auer rods
RAEB 2: 10-19% blasts, +/- auer rods
(both can be uni or multilineage dysplasa)
question
what is 5q-?
answer
a unilineage myelodysplasia, <5% blasts and no auer rods. lenalidamide specifically approved by FDA to treat this
question
how are myelodysplasic syndromes diagnosed?
answer
blood count, if low, could be MDS due to anemia, neutropenia, thrombocytopenia, or any combo - so do bone marrow bx and look for dyplastic features. cytogenetics should also be assessed.
question
how doe myelodysplastic syndromes present clinically?
answer
macrocytic anemia, low retic count (BM not working), normal to high EPO (high because body is trying to stimulate more RBCs). B12/folate deficiency needs to be ruled out esp if macrocytic
question
how do myelodysplastic syndromes appear in a bone marrow bx?
answer
megaloblastic erythroid cells, ringed sideroblasts, micromegakaryocytes, and large/giant dysfunctional platelets
question
how are myelodysplastic syndromes treated?
answer
there is no standard of care, supportive care: transfusions, growth factors (EPO, G-CSF, GM-CSF, IL-11), chemotherapy, (azcitidine, lenalidomide, amfostine)
question
how does azacitidine work?
answer
azacitidine treats MDS by DNA hypomethylation, which restores normal function of differentiation/proliferation. it is directly cytotoxic. can double 2 yr survival.
question
what is lenalidamide?
answer
a thalidomide analogue that has no sedation or neuropathy associated with it. this improved RA in 64% of pts in an 8 wk study and will also work for 5q-
question
what is a caution for giving pts repeated transfusions?
answer
the body has no way of getting rid of iron, so they can get iron overload
question
what drug can help with sideroblast forms of RA?
answer
high dose pyridoxine
question
what MDS has the lowest likelihood of leukemia? what MDS has the highest likelihood of leukemia?
answer
lowest: RA or RARS - b/c lowest blast level. highest: RAEB II
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