What does RC-UD stand for?
refractory cytopenia with unilineage dysplasia
Who gets MPNs?
What is the later stage of PV called, and what characterizes it?
spent phase; prominent fibrosis of the marrow and falling peripheral blood cell counts
What does high grade MDS mean?
myeloblasts account for >5% of marrow cells and/or more than 2% of peripheral blood cells
What is the prognosis of RC-MD? Survival rate? Transformation?
poor- median survival 2.5 years- 10% transformation to AML
What is RC-MD?
low grade MDS with dysplasia in 2 or more lineages
In the ____ stage of PMF, the marrow is hypercellular, showing a granulocytic and megakaryocytic proliferation.
What is the prognosis for refractory anemia with excess blasts-2 (RAEB-2)? Median survival? Transformation?
poor; 9 mos; 33% of patients transform to AML
What are the treatments of PV?
serial phlebotomy; aspirin; mild chemo
What is MDS with isolated deletion 5q?
low grade MDS assoc. with anemia, increased platelets, and megakaryocytes with small, round, non-lobated nuclei
What is refractory anemia with excess blasts-1 (RAEB-1)?
5-9% blasts in the marrow or 2-4% blasts in the peripheral blood
Nearly all cases of PV have a mutation of the _____ gene that encodes a _____.
JAK2; JAK2 cell signaling protein
What is the intermediate phase of CML?
the accelerated phase
What are the 4 main types of MPNs?
1. chronic myelogenous leukemia (CML) 2. polycythemia vera (PV) 3. primary myelofibrois (PMF) 4. essential thrombocytopenia (ET)
What are the 3 types of low grade MDS?
1. refractory cytopenia with unilineage dysplasia (RC-UD) 2. refactory cytopenia with multilineage dysplasia (RC-MD) 3. MDS with isolated deletion 5q
What does ET stand for?
What does RC-MD stand for?
refactory cytopenia with multilineage dysplasia
What does MPN stand for?
What does dyserythropoeisis look like?
RBC precursors with nuclear budding, irregular shaped nuclei, lack of coordinated nuclear/cytoplasmic maturity, increased ring sideroblasts
What is refractory anemia with excess blasts-2 (RAEB-2)?
10-19% blasts in the marrow or 5-19% blasts in the peripheral blood
What are dacrocytes?
tear drop RBCs
What are the s/s of CML?
fatigue, weight loss, night sweats, splenomegaly, anemia
What is the prognosis of RC-UD?
What are the s/s of ET?
TIAs; digital ischemia with parasthesias; thrombosis of major BVs
What does low grade MDS mean?
myeloblasts account for
What is the treatment for MDS?
stem cell transplant, supportive care, transfusions
What is the prognosis of PV? Survival?
very good- 10-20 years
What is MDS?
hematopoietic stem cell disorder characterized by ineffective hematopoiesis and increased risk of transformation to acute myeloid leukemia (AML)
____ should always be considered in a pt with a thrombosis of the mesenteric, portal, or splenic vein.
MPNs are usually associated with _____ that encode cytoplasmic or receptor protein tyrosine kinase (PTKs).
abnormalities of genes (translocations, point mutations)