WBC Part 7 Flashcards
(43 cards)
Myelodysplastic Syndromes (MDS) are abnormalities in what that result in what
clonal multipotent stem cell abnormality resulting in ineffective hematopoiesis with dysplasia
what type of patient would one expect to see myelodysplastic syndrome in
older patient with refractory pancytopenia or macrocyric anemia
someone who had radiation or chemo 2-7 years prior
what does one see in the peripheral blood of a patient with mylelodysplastic syndromes (MDS)
pancytopenia with macrocytic anemia
dysplastic neutrophils
what does one see in the bone marrow of a patient with mylelodysplastic syndromes (MDS)
packed marrow
trilineage hyperplasia with dysplasia in at least one line
increased storage iron
blasts less than 20%
ineffective hematopoiesis with dysplasia is seen in what
myelodysplastic syndromes
mylelodysplastic syndromes (MDS) prognosis depends on what
% blasts
amount of dysplasia
complex cytogenetics
therapy related
what is the most common cytogenetic change seen in mylelodysplastic syndromes (MDS)
monosomy (5 or 7)
del5q, del7q, del20q
trisomy 8
chromosomal abnormalities seen in about 50% of MDS
Myelodisplastic syndromes are a group of disorders, what are the high and low grade disorders
Refractory Anemia (low grade) Refractory Anemia with Excess Blasts (high grade)
low grade MDS is diagnosed how
exclusion
MDS can progress to what
AML
how are myelodisplastic syndromes treated
young patient- allogenic BM treatment
older patient- supportive therapy
Chronic Myeloproliferative Diseases (CMPD) is characterized by what kind of hematopoiesis
effective hematopoiesis just too much of it
can give rise to extramedulary hematopoiesis
Chronic Myeloproliferative Diseases (CMPD) have what associated cellularity in the BM
hypercellularity with associated increased peripheral sounds
no dysplasia
What conditions are Chronic Myeloproliferative Diseases (CMPD)
Chronic Myeloid Leukemia (CML) Polycythemia Vera (PV) Essential Thrombocytopenia (ET) Primary Myelofibrosis Chronic Eosinophilic Leukemia/Hypereosinophilic Syndrome
Chronic Myeloid Leukemia (CML) is what mutation and in what type of cell
pluripotent stem cell
t(9;22) BCR-ABL fusion gene
how can one ID the BRC-ABL gene
FISH or PCR
what is seen in the peripheral blood of CML patient
leukocytosis and neutrophilic with left shift
eosinophilia and basophilia
thrombocytosis in 50%
what is seen in the BM of a CML patient
packed marrow with prominent granulocytic hyperplasia
many small megakaryocytes
what happens to CML if not treated
slow progression to AML/ALL
what drug inhibits BCR-ABL kinase activity
Gleevac
in order to diagnose polycythemia vera what must be ruled out
secondary polycythemia
polycythemia vera is an issue with what cells and due to what mutation in 95% of cases
multipotent stem cell
JAK-2 mutation
what is seen in the peripheral blood of a polycythemia vera patient
decreased serum erythropoietin
erythrocytosis, leukocytosis, thrombocytosis
basophilia
what is seen in the bone marrow of polycythemia vera patient
hyper cellular with trilineage
no dysplasia
minimal reticulin fibrosis and EMH (late stage can get it)
