Krafts- CML Module Flashcards
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?
CML
PV
ET
MF
CMPDs occur only in________and have a __________course.
adults
LONG
What are the common features of CMPDs?
Hypercellular marrow
Big spleen–d/t extramedullary hematopoeisis
Mutated TRKs
May evolve into
CMPDs may evolve into…..
Acute leukemias
What are the common features associated w/ CML?
Philadelphia chromosome t(9;22)–>
Neutrophillic leukocytosis
Basophilia
*Three phases of clinical disease
Elevated WBC Neutrophilia w/ left sheft basophilia decreased Hb Increased platelets decreased LAP
are characteristic lab findings of what leukemia?
CML
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?
CML!
**he may also have some lymphadenopathy
What are the clinical phases of CML?
SLOW ONSET
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?
cytogenetics
PCR
Polycythemia vera is most commonly associated with what?
HIGH RBC
What clinical sx are associated w/ PV?
Thrombosis and hemorrhage
What mutation is associated w/ PV?
Jak-2
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
splenomegaly
How does a mutated JAK 2 cause PV?
Mut JAK-2>
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?
PV
dizziness, thrombosis, infarction
phlebotomy
mS drugs
What disease has a VERY HIGH platelet count in blood and often occurs in young women?
Essential thrombocytothemia
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?
ET
Platelet pheresis
MS drugs
aspirin
Chronic myelofibrosis is characterized by _______followed by ____________.
Panmyelosis (many cells of all types)
Marrow fibrosis
Many CMPDs have this but it is often more so in CMF than others.
Extramedullary hematopoiesis
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.
