CML
Polycythemia Vera
Essential Thrombocyosis
Primary Myelofibrosis
Therapeutics for MPNs
100
This city is the namesake of the classic t(9,22) found when parts of chromosomes 9 and 22 broke off and fused.
What is Philadelphia?
100
This symptom is a common complaint of patients with P vera.
What is pruritus?
100
Platelet count > _____ is typical of ET
What is 1,000,000?
100
"Leukoerthroblastic" refers to the appearance on a ______.
What is blood smear?
100
Gleevec (imatinib mesylate) was the first such drug to inhibit this protein.
What is BCR-ABL?
200
The fusion protein is a combination of parts of which proteins.
What is BCR and ABL?
200
Many patients present with _______ as a serious complication of P vera.
What is thrombotic episode?
200
Risk for thrombosis is highest in patients older than ____.
What is age 60?
200
Primary myelofibrosis is difficult to differentiate from ________ myelofibrosis.
What is secondary?
200
Without _____ patients with P vera may only live less than a couple years, rather than over a decade on average.
What is phlebotomy? Baby aspirin is also indicated for these patients. Hydrea can be used to supplement phlebotomy but is "leukomogenic" and used later in the disease.
300
BCR-ABL is overactive and subsequently phosphorylates proteins in this class.
What are tyrosine kinases?
300
P vera patients live for decades. Their disease can transform to _____.
What is AML?
300
JAK2 is mutated in ____% of patients with ET.
What is ~50%?
300
Enlargement of this organ is very common in PMF.
What is the spleen?
300
A patient presents with elevated WBC, splenomegaly and is ill. BCR-ABL is positive and many blasts are seen on smear consistent with accelerated phase. A second generation TKI is given called _______.
What is dasatinib?
400
The most common phase at presentation of CML is _____.
What is chronic phase?
400
Enlarged spleen, elevated red cell mass, normal lung function and low erythropoitin level are helpful in diagnosing P vera. The mutation ______ is present in almost 95% of patients with P vera.
What is JAK2?
400
Parodoxically, _____, can occur in ET patients.
What is bleeding?
400
JAK2 is also found to be mutated in a percentage of patients with PMF. They are now able to be treated with ______ inhibitors.
What are JAK 1 and 2 inhibitors? Ruxolitinib is the first such one approved by the FDA
400
Prior to ruxolitinib patients with PMF or the fibrosis phase of their myeloproliferative disorders were treated with _______ instead of pharmacotherapy.
What is supportive care? Androgens, steroids, thalidomide, interferon have been tried and are largely not helpful. Splenectomy can be done for palliation, but is fraught with complications, among them estramedullary hematopoiesis.
500
A patient presents feeling well with elevated WBC count > 50,000, predominance of leukocytes on smear and an enlarged spleen. Diagnosis can be made by sending his blood for ______.
What is qualitative PCR for BCR-ABL?
500
Polycythemia vera is best treated by _____.
What is phlebotomy to maintain a HCT < 50?
500
A 75 yo patient presents with platelets>1,000,000 and a CVA. Serum ferrittin is normal, no infection is present and the patient's spleen is intact. Besides starting aspirin, you should start _____.
What is hydroxyurea 500mg and titrate to keep platelets < 600,000? Hydrea was shown to be safer longer term with less thromboses in a head to head trial with anegralide.
500
A patient presents with profound fatigue, splenomegaly and pancytopenia. Bone marrow biopsy is consistent with PMF with negative JAK2, BCR-ABL and no metastatic carcinoma seen. The patient has a _____ risk of transformation to AML than a patient with ET?
What is higher?
500
Curing MPN can be done in rare situations with ______
What is allogeneic stem cell transplant? Generally reserved for younger patients of good performance status.
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