1. What statement is TRUE regarding triple negative disease in myelofibrosis (MF)?
A. Patients do not present with classic MF symptoms of anemia, fatigue, and splenomegaly
B. It is a positive risk factor and is associated with the best outcomes in MF
C. The common driver mutations of JAK2, CALR, and MPL are not present
D. The common driver mutations of ASXL1, SRSF2, and U2AF1Q157 are not present
E. Unsure
2. With which symptoms do MF patients most frequently present that affects their quality of life?
A. Neuropathy and night sweats
B. Anemia and fatigue
C. Hair loss and fatigue
D. Splenomegaly and anemia
E. Unsure
3. Which statement regarding the risk stratification scoring systems in MF is TRUE?
A. The Dynamic International Prognostic Scoring System (DIPSS) can only be used at the time of diagnosis
B. A score of 4 to 9 on the Mutation-enhanced International Prognostic Scoring System Version 2 (MIPSS70+v2) is considered lower-risk disease
C. Anemia severity values are the same for males and females in the MIPSS70+v2 scoring system
D. MIPSS70+v2 is a 5-tiered system using molecular, karyotype, and clinical variables to determine risk
E. Unsure
4. The physician you are working with in clinic wants to start immunomodulatory inhibitor (IMiD) therapy for her patients with MF-associated anemia. What information would you provide her regarding this therapy?
A. To mitigate thalidomide toxicity, it can be given with a prednisone taper initiated at 0.5 mg/kg/day
B. Pomalidomide is the treatment of choice, as it has the best outcomes of the 3 available IMiDs
C. Lenalidomide is the best tolerated IMiD, as it showed a low incidence of myelosuppression in clinical trials
D. If her patient has a del 5q, then thalidomide is the drug of choice over other IMiDs
E. Unsure
5. The oncologist you are working with decides to initiate hydroxyurea to treat his patient's MF-associated splenomegaly. Which of the following is the most appropriate counseling point for the patient?
A. Pay careful attention to and report any changes in skin integrity, as hydroxyurea can cause lower extremity ulcerations
B. You will most likely be on hydroxyurea therapy for an extended period of time, as the median duration of response is 36 months
C. Report any mood changes to your oncologist, as hydroxyurea treatment can exacerbate or cause depression
D. Once you are on a stable dose of hydroxyurea, you will only need blood draws to check for cytopenias every 6 months
E. Unsure
6. SJ has been on ruxolitinib therapy for 1 year at a dose of 25 mg twice daily. At his next clinic visit his platelets are 75 x 109/L. What is the proper management of SJ's therapy going forward?
A. Continue ruxolitinib at the current dose and recheck platelets in 2 to 4 weeks
B. Decrease the ruxolitinib dose to 5 mg twice daily and recheck platelets in 2-4 weeks
C. Decrease the ruxolitinib dose to 10 mg twice daily and recheck platelets in 2-4 weeks
D. He is experiencing toxicity that warrants a change in therapy; switch to fedratinib
E. Unsure
7. Which of the following is FALSE regarding the monitoring and management of fedratinib therapy?
A. The risk of Wernicke's encephalopathy (WE) is a Boxed Warning with fedratinib
B. WE with fedratinib can include symptoms of ataxia, mental status changes, and confusion
C. Thiamine deficiency must be corrected prior to fedratinib therapy initiation to prevent WE
D. If WE is suspected, the fedratinib dose should be reduced to 100 mg daily
E. Unsure
8. The oncologist you are working with in clinic wants to change his patient from ruxolitinib to fedratinib due to lack of response to ruxolitinib therapy. Which of the following is FALSE regarding managing this change?
A. A slow taper of ruxolitinib is the best way to prevent ruxolitinib withdrawal syndrome
B. Patients at highest risk of ruxolitinib withdrawal syndrome are those with a high dose, low platelets, and a large spleen size at the time of discontinuation
C. If ruxolitinib withdrawal syndrome occurs, management strategies include the initiation of systemic steroids and treatment of any tumor lysis syndrome or hyperproliferative symptoms
D. Fedratinib therapy should overlap with ruxolitinib therapy to prevent withdrawal syndrome
E. Unsure
9. Which of the following best describes luspatercept's mechanism of action?
A. Inhibits JAK 1 and 2, thereby inhibiting cell signaling via the JAK-STAT pathway
B. Stops transforming growth factor-β ligands from binding to activin IIB receptors
C. Directly stimulates erythropoietin progenitor cells
D. Inhibits ACVR1/ALK2 which upregulates hepcidin synthesis to ameliorate anemia
E. Unsure
10. HT is currently well controlled on therapy with fedratinib 400 mg orally daily for her MF diagnosis. She requires treatment with voriconazole (a strong CYP3A4) inhibitor with no other option. What is the best management strategy for HT's MF therapy?
A. Change fedratinib to ruxolitinib and restart fedratinib when the voriconazole is discontinued
B. Reduce the fedratinib dose to 200 mg orally daily and return to 400 mg orally daily when the voriconazole is discontinued
C. Reduce fedratinib dose to 200 mg orally daily, then increase to 300 mg orally daily for 2 weeks, then 400 mg orally daily thereafter when the voriconazole is discontinued
D. Reduce the fedratinib dose to 300 mg orally daily and return to 400 mg orally daily when the voriconazole is discontinued
E. Unsure
Evaluation Questions
11. How confident are you in your treatment choice for SJ in question #6 above?
A. Not at all confident
B. Somewhat confident
C. Confident
D. Highly confident
12. How confident are you in your treatment choice for HT in question #10 above?
A. Not at all confident
B. Somewhat confident
C. Confident
D. Highly confident