Myelodysplastic Syndrome and Myeloproliferative Neoplasms Flashcards
MDS presents with ________.
ineffective hematopoiesis due to dysplastic growth of a clone; persistent peripheral cytopenia of multiple cell lineages; median age of 70
Chromatin will often look _______ in dysplastic myeloid cells.
“moth-eaten”
What are the three classifications of myeloproliferative disorders?
low grade MDS (20% myeloblasts)
Myeloproliferative neoplasms are characterized by __________.
an increase in one line of functional hematopoietic cells
CML is defined by _________.
the presence of the BCR-ABL1 translocation
When CML enters the “blast phase,” it is then considered to be _______.
AML
Polycythemia vera is characterized by ________.
erythrocytosis, increases in other myeloid cells
Other than PV, what patients might have erythrocytosis?
Smokers, those who live at high altitude, those with high-affinity hemoglobin
Under morphologic examination, what should an MDS patient’s blood demonstrate?
Erythrocyte precursors with abnormal nuclei and ring sideroblasts; granulocyte precursors with hypogranulation
What karyotypic findings are suggestive of MDS?
Monosomy of 5 or 7; q deletions of 5 or 7; trisomy 8
Prior to diagnosing someone with MDS, you should exclude cytopenias from ______.
toxin exposure, anemia, and nutrient deficiency
What are typical prognoses for MDS?
Low grade has a median survival of either over five years (for one unilineage) or 2.5 years, and high grade has a survival 16 months or 9 months
Those with myeloproliferative disorders have bone marrow dysfunction such as ________.
fibrotic scarring, ineffective hematopoiesis, or transition to AML
Which disorders have hepatosplenomegaly?
MPNs
What are the four main types of MPNs?
Polycythemia vera, essential thrombocythemia, CML, and primary myelofibrosis
What is the typical presentation age for CML?
40 - 50
What was the second-generation Gleevec called?
Dasatinib
Polycthemia vera should be considered in those with _________.
emboli in the splenic vein, mesenteric vein, or portal vein
“Pins-and-needles” are medically referred to as ________.
paresthesias
PV has a ________ prognosis.
good (10-20 year survival)
Primary myelofibrosis is like PV but without ________.
erythrocytosis
Essential thrombocythemia and primary myelofibrosis both have ______ mutations.
JAK2
