Myelodysplastic Syndrome and Myeloproliferative Neoplasms Flashcards
Clonal stem cell disorders with defective maturation and ineffective hematopoiesis
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Myelodysplastic Syndrome (MDS)
Myelodysplastic Syndrome (MDS)
What lab values are associated?
What would the bone marrow look like?
Myelodysplastic Syndrome (MDS)
pancytopenia
hypercellular bone marrow
a deletion of chromosome 5q can lead to what syndrome?
Myelodysplastic Syndrome (MDS)
you might also see
+8 (trisomy 8)
-7 (del 7q)
These are characteristic cells found in bone marrow aspirate in a patient with myelodysplasia
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Ringed sideroblasts are erythroid progenitors seen in myelodysplasia
These cells are characteristicly found in a periphereal blood smear of a patient with?
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Pseudo-Pelger-Huet cells are neutrophils with only 2 nuclear lobes instead of the normal 3-4.
they are seen in the peripheral blood smear of a patient with Myelodysplasia
What genetic variation is associated with a better prognosis in MDS?
isolated deletions in 5q is assciated with a better prognosis in MDS.
It has a better response to thalidomide
Chronic Myeloid Leukemia (CML)
mutation:
Chronic Myeloid Leukemia (CML)
t(9;22)
BCR-ABL1 known as the Philadelphia chromosome
Chronic Myeloid Leukemia (CML)
LAP and NAP lab values will be (low/high)?
Leukocyte alkaline phosphate or neutrophil alkaline phosphate levels will be
LOW
Chronic Myeloid Leukemia (CML)
Treatment
imatinib
a tyrosine kinase inhibitor
This is associated with in increase in red cell production and is more common in Ashkenazi Jews.
Mutation of JAK2
Polycythemia Vera (PVC)
Polycythemia Vera (PVC)
would our serum erythropoeitin be (high, low, normal)?
decreased
Polycythemia Vera (PVC)
Treatment
Polycythemia Vera (PVC)
Phlebotomy
myelosuppression with hydroxyurea, IFN-alpha, ruxolitinib
Polycythemia Vera (PVC)
in what phase does massive splenomegaly occur?
Polycythemia Vera (PVC)
massive splenomegaly occurs in the spent phase of PVC because of the extramedullary hematopoiesis occurring in the setting of advanced marrow myelofibrosis
JAK2 mutations or MPL mutations
sustained platelet counts 450/microL
no polycythemia or marrow fibriosis
Essential Thrombocytothemia
thrombopoietin-independent thrombocytosis
this peripheral blood smear shows marked thrombocytosis including giant platelets
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Essential Thrombocythemia