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Flashcards in CML Module - Krafts Deck (36)
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Chronic leukemias involve:

More mature lymphocytes


What things must you know about chronic myeloproliferative disorders?

-Malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood, bone marrow [not blocked at a certain stage]
-Four disorders: CML, PV, ET, MF
-Occur only in adults
-Long course (could live a few years without treatment)


What are the names of the two chronic leukemias?

These are not benign, they're leukemias!!
1. Chronic myeloproliferative disorders (four)
2. Chronic lymphoproliferative disorders


What are the four chronic myeloproliferative disorders?

1. Chronic myeloid leukemia (neutrophils predominating)
2. Polycythemia vera (red cells predominating)
3. Essential thrombocytopenia (not throbocytosis! - megakaryocytic/platelet predominating)
4. Myelofibrosis (everything proliferating same amount, then it becomes fibrinoid)


What is unique about chronic myeloproliferative disorders?

-They include mostly mature cells, but can also include some of the lesser developed upper cells in the myeloid lineage


What is proliferating most in the myeloproliferative disorders?

-CML - neutrophils
-PV - red cells
-ET - platelets
-MF - everything!


What features are common to all four myeloproliferative disorders?

-Occur only in adults
-Long clinical course
-Inc. WBC with left shift (usually seen in routine CBC)
-Hypercellular marrow
-Big spleen
-May evolve into acute leukemia (almost all of these - as an end stage event, process changes from chronic to acute)
-Mutated tyrosine kinases (CML and other myeloproliferative disorders - mutated thing telling itself to grow all the time)


What MUST you know about Chronic Myeloid Leukemia? (EXAM!!)

-Neutrophilic leukocytosis
-Philadelphia chromosome (EXAM!!)
-Three phases


What do you see in slides of CML?

-Abundant mature neutrophils
-Some myelocytes, promyelocytes, blasts
-BASOPHILIA - multiple basophils in a field


What are the laboratory findings in CML?

-VERY HIGH WBC count (from malignant cells)
-Neutrophilia with left shift (more malignant early cells)
-Low hemoglobin
-High platelet count (at first) (malignant platelets, normal platelets are decreased in number)
-Dec. LAP (leukocyte alkaloid phosphatase - enzyme present in neutrophils - if bening, LAP should be increased)


What chromosomal abnromalities are found in CML?

-Translocation between chromosome 9 and 22
-Chromosome 22 - Philidelphia chromosome - where the action happens
-BCR-ABL hybrid gene --> tyrosine kinase


What are the clinical findings (symptoms & signs) in CML?

Symptoms (release of enzymes makes patients sick: fever, night sweats from too many cytokines!):
-Slow onset
-Fever, fatigue, night sweats
-Abdominal fullness
-Big spleen
-Big liver


What is the chronic phase of CML like?

Most patients present with this, but how they pass through the phases is random in timeline and length
-Stable counts
-Marrow full of cells
-Neutrophil series
-Hemoglobin decreased, platelets up
-Easily controlled (with drugs)
-3-4 years (untreated)


What is the accelerated phase of CML?

50% of chronic phase patients go onto accelerated phase.
-Unstable counts
-Blast crisis within 6-12 months
-Things are changing quickly
-Not much you can do for patient
-Patients usually die in this phase or when they go into blast crisis


What is the blast crisis phase of CML?

50% of chronic phase patients do onto blast phase. It is extremely difficult to treat and patient likely dies in a month or so.
-Acute leukemia
-More than 20% blasts in blood
-Blasts can be either myeloid or lymphoid!
-High mortality


What is CML remission like?

Hematologic Remission
-No splenomegaly
-WBC extremely sensitive


What MUST you know about Polycythemia Vera?

-High RBC (makes blood sludgy)
-Different from secondary polycythemia
-Thrombosis and hemorrhage
-Jak-2 mutation


What are the types of Polycythemia Vera? (EXAM!?)

"Polycytemia" = Inc. red cell mass
-Primary (intrinsic myeloid cell problem - low erythropoietin)
-Secondary (due to increased erythropoietin)


What are the clinical findings in PV?

-Headache, pruritis, dizziness
-Thrombosis, infarction (clotting and bleeding tendency - something wrong with platelets and the way they interact)
-Big spleen, liver
-Plethora (flushing of face and neck)


What does Polycythemia Vera look like on a slide?

-Red cells are right next to each other/on top of each other
-Bone marrow is hyper cellular
-Erythroid series is predominant


What is different about the JAK-2 in PV?

Normal JAK-STAT pathway
--Cell signaling pathway
--Important in many different cell types
--Mutated JAK-2; activity increased in PV (JAK activating STAT all the time)
--Cells grow on their own
-Important for diagnosis and drug therapy
-95% of PV have this mutation


What is the treatment for PV?

-Maybe myelosuppressive drugs


What is the prognosis for PV?

-Median survival: 9-14 years
-Death from thrombosis or hemorrhage
-Leukemic transformation in some patients


What things MUST you know about essential thrombocythemia?

-Very high platelet count in blood
-Can occur in young women (still adults) - many other diseases peak in 50s or 60s
-Diagnosis of exclusion
-Thrombosis and hemorrhage


What is the diagnostic criteria for ET?

-Need to make sure platelet count is REALLY HIGH
-Platelet count >600,000


What are the clinical features of ET?

-Purpura, bruising
-Pallor, tachycardia
-Biggish spleen


What do you see in the slide (blood) and bone marrow of essential thrombocythemia?

-Lots of platelets here
-Hypercellular bone marrow


What is the treatment of ET?

-Platelet pheresis
-Maybe myelosuppressive drugs


What is the prognosis of ET?

-Median survivial: 5-8 years
-Death from thrombosis or hemorrhage
-Leukemic transformation in some patients


What things MUST you know about Chronic Myelofibrosis?

-Panmyelosis. . . (all myeloid lines are proliferating at once)
-. . .then marrow fibrosis
-Extramedullary hematopoiesis (VERY MASSIVE FOR CM)
-Teardrop red cells