5-23 KRAFTS CML Flashcards
What are the 4 things you must know about Chronic Myeloproliferative Disorders?
Malignant proliferation of maturing myeloid cells in blood/BM
Four disorders: CML, PV, ET, MF
Occur almost only in adults
Long course (Chronic!)
What is proliferating most in CML, PV, ET, and MF?
Chronic myeloid leukemia – Neutrophils
Polycythemia vera – RBCs
Essential thrombocythemia – Platelets
Myelofibrosis – EVERYTHING
Why do we see splenomegaly in all of these disorders?
B/c additional hematopoiesis
What are the 4 things you must know about Chronic Myeloid Leukemia?
↑Neutrophils
↑Basophils!
Philly chrome
3 phases (chronic accelerated blast crisis)
What is LAP?
Leukocyte alkaline phosphatase, a N.phil enzyme (decreased in CML)
What are the different kinds of remission you could see in CML?
Hematologic remission – WBC, Hgb, platelets all normal
Cytogenetic remission – No Philly chrome
Molecular remission – No BCR/ABL transcripts via PCR
What are the 4 things you must know about Polycythemia Vera?
↑RBC
Different from secondary polycythemia (which is due to ↑EPO)
Thrombosis & hemorrhage
Jak-2 mutation (necessary for diagnosis)
What are some symptoms of PV?
Big spleen, liver, Plethora (ruddy face)
Tx of PV?
Phelbotomy, myelosuppressive drugs
What are the 4 things you must know about Essential Thrombocythemia?
↑↑↑platelet count!
Can happen in young ♀
Diagnosis of exclusion
Thrombosis and hemorrhage
Why is ET a Dx of exclusion?
B/c all the other disorders can have ↑platelet count
Tx for ET?
Platelet pheresis, myelosuppressive drugs, aspirin
What can ET transform into?
Leukemic transformation can result in AML or ALL. Shitty.
What are the 4 things you must know about Chronic Myelofibrosis (MF)?
Panmyelosis
FOLLOWED BY marrow fibrosis
Extramedullary hematopoiesis ( splenomegaly)
Teardrop red cells
Why do we get the teardrop red cells?
B/c they’re trying to squeeze through fibrotic tissue in BM to get to blood
