Myeloproliferative Neoplasms (MPNs) Rounds Pre-Assessment
This activity is provided by The Leukemia & Lymphoma Society and Medical Learning Institute Inc, in collaboration with the Association of Cancer Care Centers™ (ACCC).
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Pre-Assessment Responses
1. Which goal of therapy with a JAK inhibitor is not an intended goal?
Symptom control
Spleen reduction
Improved survival
Anemia improvement
Platelet normalization
2. What is the preferred first line of treatment for a "low-risk" JAK2 ET patient with symptoms?
Aspirin alone and cardiovascular risk reduction
A + Interferon alpha
A + Hydroxyurea
3. 67 yo M with PMF diagnosed 6 months ago, who was started on ruxolitinib at the time of diagnosis due to palpable spleen 20 cm and significant symptoms including debilitating fatigue, weight loss, early satiety, and bone pains (MPN-SAF TSS = 28). He had a good response to ruxolitinib 15mg BID with resolution of many of his symptoms and a now nonpalpable spleen (MPN-SAF TSS = 4). Over the past month, he has become more anemic and is experiencing new dyspnea on exertion. He is otherwise asymptomatic. Labs: WBC 9.2, Hgb 7.5, MCV 90, plt 125, LDH 250, EPO 700. Iron panel, vitamin B12, and folate are within normal limits. What is the next best step in management of this patient?
Change from current therapy to pacritinib
Change from current therapy to momelotinib
Add lenalidomide to current therapy
Add luspatercept to current therapy
Add danazol to current therapy
4. A 50 year old woman with advanced systemic mastocytosis continues to experience fatigue, dyspepsia, diarrhea, palpitations, and neurocognitive complaints despite her religious use of H2 blocker, corticosteroids, cromolyn, PPI. She has lytic bone lesions and is on a bisphosphonate. She is anemic and her platelets are 42k. What treatment do you offer her if any?
Watch and wait
Avapritinib
Midostaurin
Beclastinib
Cladribine
5. A 45 year old man presents with fatigue and left upper quadrant discomfort. On examination his spleen is 8 centimeters below the left costal margin. His white blood count is 328,000/mm3 with 33% neutrophils, 2% lymphocytes, 6% monocytes, 2% eosinophils, 7% basophils, 6% bands, 9% metamyelocytes, 23% metamyelocytes, 4% promyelocytes and 8% blasts. The hemoglobin is 7.4 gm/dl and the platelet count is 75,000/mm3. Bone marrow evaluation demonstrates that the patient has CML in chronic phase. Which of the following worsens the patient’s prognosis relative to other CML patients.
Young age
Markedly elevated white blood count (328,000/mm3)
Hemoglobin 7.4 gm/dl
Platelet count 75,000/mm3
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Post-Assessment Responses
Post 1. Which goal of therapy with a JAK inhibitor is not an intended goal?
Symptom control
Spleen reduction
Improved survival
Anemia improvement
Platelet normalization
Post 2. What is the preferred first line of treatment for a "low-risk" JAK2 ET patient with symptoms?
Aspirin alone and cardiovascular risk reduction
A + Interferon alpha
A + Hydroxyurea
Post 3. 67 yo M with PMF diagnosed 6 months ago, who was started on ruxolitinib at the time of diagnosis due to palpable spleen 20 cm and significant symptoms including debilitating fatigue, weight loss, early satiety, and bone pains (MPN-SAF TSS = 28). He had a good response to ruxolitinib 15mg BID with resolution of many of his symptoms and a now nonpalpable spleen (MPN-SAF TSS = 4). Over the past month, he has become more anemic and is experiencing new dyspnea on exertion. He is otherwise asymptomatic. Labs: WBC 9.2, Hgb 7.5, MCV 90, plt 125, LDH 250, EPO 700. Iron panel, vitamin B12, and folate are within normal limits. What is the next best step in management of this patient?
Change from current therapy to pacritinib
Change from current therapy to momelotinib
Add lenalidomide to current therapy
Add luspatercept to current therapy
Add danazol to current therapy
Post 4. A 50 year old woman with advanced systemic mastocytosis continues to experience fatigue, dyspepsia, diarrhea, palpitations, and neurocognitive complaints despite her religious use of H2 blocker, corticosteroids, cromolyn, PPI. She has lytic bone lesions and is on a bisphosphonate. She is anemic and her platelets are 42k. What treatment do you offer her if any?
Watch and wait
Avapritinib
Midostaurin
Beclastinib
Cladribine
Post 5. A 45 year old man presents with fatigue and left upper quadrant discomfort. On examination his spleen is 8 centimeters below the left costal margin. His white blood count is 328,000/mm3 with 33% neutrophils, 2% lymphocytes, 6% monocytes, 2% eosinophils, 7% basophils, 6% bands, 9% metamyelocytes, 23% metamyelocytes, 4% promyelocytes and 8% blasts. The hemoglobin is 7.4 gm/dl and the platelet count is 75,000/mm3. Bone marrow evaluation demonstrates that the patient has CML in chronic phase. Which of the following worsens the patient’s prognosis relative to other CML patients.
Young age
Markedly elevated white blood count (328,000/mm3)
Hemoglobin 7.4 gm/dl
Platelet count 75,000/mm3
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