Flashcards in Krafts- CML Module Deck (26):
What are chronic myeloproliferative disorders?
Malignant proliferation of myeloid cells (NOT blasts, maturingn cells) in blood and bone marrow?
What are the 4 disorders associated w/ CMPDs?
CMPDs occur only in________and have a __________course.
What are the common features of CMPDs?
Big spleen--d/t extramedullary hematopoeisis
May evolve into
CMPDs may evolve into.....
What are the common features associated w/ CML?
Philadelphia chromosome t(9;22)-->
*Three phases of clinical disease
Neutrophilia w/ left sheft
are characteristic lab findings of what leukemia?
A 57 y/o M presents to you w/ fever, fatigue, night sweats and says he feels a dragging sensation in his belly. Upon palpation his liver is enlarged.
What's wrong with him?
**he may also have some lymphadenopathy
What are the clinical phases of CML?
chronic- stable counts, easily controlled
accelerated- unstable counts
blast crisis- lots of blasts
What translocation is associated w/ CML and what does it do?
t(9;22)--> BCR-ABL hybrid gene--> codes for a NASTY TRK
How do you dx CML?
Polycythemia vera is most commonly associated with what?
What clinical sx are associated w/ PV?
Thrombosis and hemorrhage
What mutation is associated w/ PV?
What are the two types of PV?
Primary- Intrinsic myeloid cell problem
Seconadry- d/t increased EPO
What are the three main criteria to diagnose PV?
Increased red cell mass
normal O2 sat
How does a mutated JAK 2 cause PV?
inability to inhibit JAT-STAT pathway>
excessive cellular growth w/o external signal
A pt presents w/ HA, pruritis, a big spleen and plethora. What might they have? What other sxs may you be conerned about? How do you treat them?
dizziness, thrombosis, infarction
What disease has a VERY HIGH platelet count in blood and often occurs in young women?
ET is often a diagnosis of exclusion. What lab findings would rule it out.
NO philadelphia chromosome
NO marrow fibrosis
NO other reasons for thrombocytosis
Platelet count > 600,000
Hgb<13 or RBC mass normal
A pt presents w/ bleeding, thrombosis, tachycardia, bruising and a big spleen. What might they have? How do you tx them?
Chronic myelofibrosis is characterized by _______followed by ____________.
Panmyelosis (many cells of all types)
Many CMPDs have this but it is often more so in CMF than others.
What causes tear drop cells?
In chronic myelofibrosis, tear dropped shaped RBC are formed when RBC can't move freel through the marrow and spleen d/t the extensive fibrosis.
Pt presents w/:
weakness, fatigue, palpitations
Left upper quadrant fullness-->HUGE spleen
pallor and tachycardia
What causes these sxs?
what do they have?
how do you treat them?
Don't have red cells
MS drugs early on