Myelodysplastic/Myeloproliferative

Question 1

Myelodysplastic Syndrome

Answer 1

clonal stem cell disorder
inc risk of xsformation to AML (final stage) --> see Auer rods
accelerated apoptosis
ineffective hemato
hypercellular marrow
peripheral cytopenia

Question 2

MDS FAB classification

Answer 2

• -refractory anemia –> low RBC; normal everything else
• -refractory anemia with ringed sideroblasts –> iron ring deposits
• -refractory anemia with excess blasts –> blasts do NOT have auer rods
• -CML

Question 3

Polycythemia vera (MPD)

Answer 3

abnormal conc of RBC
inc Hb
intrinsic abnormality of myeloid stem cell
secondary: high EPO = lung disease / high altitude / cyanotic heart disease / EPO sec tumors (renal cell Ca)
HEPATOSPLENOMEGALY

Question 4

CML

Answer 4

25-60yo
t(9;22) bcr-able TK gene fusion
B and T cells show fusion
pluripotent stem cell is target
insidious onset; slow progression
splenomegaly
can undergo blast crisis --> AML

Question 5

Essential Thrombocytosis

Answer 5

Dx of exclusion
sustained platelet count > 450,000
see bleeding or thrombosis
usually past age 60
med survival 12-15yrs

Question 6

Primary Myelofibrosis

Answer 6

rapid dev of marrow fibrosis
suppression of normal hematopoiesis
extensive extramedullary hemato
collage deposition by non-neo fibroblasts
"teardrop" cells --> poikilocytes