LEC 16 Flashcards
what happens when the BM fails in myelodysplastic syndromes
no/little cells are produced
what can myelodysplastic syndromes cause
anemia, infection, and bleeding
how many cells lines are affected when the BM fails
one or more
what are the Hb and HCT values in aplastic anemia
decreased
aplastic anemia: cells values
pancytopenia
aplastic anemia: retic count
reticulocytopenia
aplastic anemia: how does the BM look
hypocellular
aplastic anemia: HSC
depleted
most cases of aplastic anemia are _____
idiopathic
def? disease that arises spontaneously and has no known cause
idiopathic
what causes secondary aplastic anemia
- drugs
- viruses
- PNH
- autoimmune diseases
what kind of aplastic anemia does fanconi anemia, dyskeratosis congenita, shwachman-bodian-diamond syndrome cause
inherited/congenital
what severity of aplastic anemia BM is hypocyelluar plus meet 2 other criteria
medium
what severity of aplastic anemia BM cellularity is less than 25% plus meets 2 other criteria
severe or very severe
what severity of aplastic anemia has 0.5 x 10^9/L to 1.5 x 10^9/L neutrophils
medium
what severity of aplastic anemia has 0.2 x 10^9/L to 0.5 x 10^9/L neutrophils
severe
what severity of aplastic anemia has less than 0.2 x 10^9/L neutrophils
very severe
what severity of aplastic anemia has 20 x 10^9/L to 50 x 10^9/L plts
medium
what severity of aplastic anemia has less than 20 x 10 ^9/L plt
severe or very severe
what severity of aplastic anemia hb less than or equal to 10 x 10^9 g/dL plus retics less than 30 x 10^9/L
medium
what severity of aplastic anemia hb less than 20 x 10^9/dL or less than 1 % corrected HCT
severe or very severe
what distinguishes severe aplastic anemia from very severe aplastic anemia
neutrophil count
what kind of gene mutation does MDS have
somatic at HSC level
t or F: cells produced in MDS function properly
F
disease?
- progressive cytopenias in PB
- abnormal cells in each line (dyspoiesis)
- one line is clonealy affected
MDS
what can MDS develop into
AML
who is likely to ge4t MDS
older
def? abnormal erythrocytes
dyserythropoiesis
disease:
- oval macrocytes
- hypo chromic microcytes
- dimorphic RBC population
MDS
what causes BM in MDS not to make enough cells
ineffective erythropoiesis
what will RBC precursors in BM in MDS look like
- more than one nucleus
- abnormal nucleus shapes
- uneven cytoplasmic staining
- ring sideroblast
def? abnormal granulocytes
dysmyelopoiesis
how will granulocytes develop in MDS
nuclear-cytoplasmic asynchrony (cytoplasm less mature)
describe the granules in neutrophils in MDS
can be granular or hypo granular
how many segments do granulocytes in MDS have
can be hypo or hyperhsegmented or ringed
what other disease does MDS get confused with
megaloblastic anemia
if neutrophils lose granularity what other cell can they be confused with in flow cytometry.
monocytes
what WBC has CD 14
monocytes
what WBC has CD 64
granulocytes
MDS classification? anemia or infection, or petechiae, less than 1% blasts in PB and less than 5% in BM
MDS with single lineage dysplasia
MDS classification? more than one lineage is dyspoietic, less than 1% blast in PB and less than 5% in BM
MDS with multi lineage dysplasia
MDS classification? SF3B1 gene mut, this mutation plus at least 5% ring sideroblast, dimorphic RBC in PBS
MDS with ring sideroblast
MDS classification? trilineage cytopenia
MDS with excess blast
MDS classification? trilineage cytopenia, 5-9% blast in BM or 2% to 4% blast in PB
MDS with excess blast 1
MDS classification? trilineage cytopenia, 10-19% blast in BM or 5% to 19% blast in PB
MDS with excess blast 2
MDS classification? affects predominantly women and occurring at a median age of 70, anemia without other cytopenias
MDS with isolated del(5q)
what is the major indication of MDS in the PB and BM
dyspoiesis
PBS findings of MDS
- oval macrocytes
- agranular neutrophils
- circulating micromegakaryocytes
a pt has anemia, oval macrocytes, and hypersegmented neutrophils. what test should be ran to ID this disorder
vit b12 and folate levels
