Internal Medicine

In a patient with no evidence of bleeding, do you use a platelet cutoff? Do you utilize genomic testing (eg CALR, MPL, JAK2, etc.) to decide on cytore...

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For patients with a JAK2 positive myeloproliferative neoplasm, do you always perform a bone marrow biopsy at diagnosis?

Despite distinguishing between the three MPNs, results may not change management. There is controversy over whether to follow WHO criteria for d...

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Under which circumstances would you prefer ropeginterferon over hydroxyurea for patients with Polycythemia Vera?

Ropeginterferon is now a preferred therapy for Polycythemia Vera (PV) as per a recent update to the NCCN guidelines.

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In which patients with essential thrombocythemia would you use ropeginterferon alfa-2b?

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For patients with essential thrombocythemia who develop venous thrombosis in the setting of elevated platelet counts, would you continue lifelong anticoagulation even after cytoreduction is achieved?

How would you consider anticoagulation vs antiplatelet therapy, or the addition of antiplatelet therapy for these patients?

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Is there a role for phlebotomy in secondary polycythemia?

Aside from addressing the underlying case, is there a role for phlebotomy in secondary polycythemia such as in COPD or post-renal transplant erythrocy...

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Is a bone marrow necessary in patient with splenomegaly and polycythemia vera?

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How would one approach concomitant diffuse large B cell lymphoma and fibrotic phase myelofibrosis?

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What is your approach to the management of essential thrombocytosis in a woman planning to start a family?

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How do you choose between peginterferon and anagrelide for ET patients if hydroxyurea-intolerant?

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Do you ever repeat screening for acquired von Willebrand in patients with essential thrombocythemia who have high platelet counts and very low risk disease not on cytoreductive therapy?

Such as in a patient with essential thrombocythemia with a CALR mutation, younger than age 60, no history of thrombosis, no bleeding or vasomotor symp...

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Is bone marrow biopsy indicated in patients with primary polycythemia but negative for JAK2 mutation including exon 12?

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Would you offer BM biopsy as the next step for progressive thrombocytosis when peripheral blood is negative for JAK2, CALR, and MPL mutations for MPN diagnosis?

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Would you use the MIPSS-PV risk scoring to decide in decision making for cytoreductive therapy in PV?

If so, would you do bone marrow biopsy or send for NGS panel in blood to look for high risk mutations? NCCN lists this as the risk criteria.

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When you send for molecular studies for polycythemia vera, what are the mutations that predict increased cardiovascular risk?

How do you use this information in clinical practice?

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How would you approach a low risk patient <60 yo with platelets <600 K, JAK2 positivity and heterozygosity for factor 5 leiden mutation with no previous thrombosis?

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Do you recommend starting an antiplatelet for primary prophylaxis in post splenectomy thrombocytosis given there is some increased risk of venous thrombosis?

If so, what platelet count threshold would you use? Would age impact your decision? Would you do a bone marrow to rule out primary MPD in adults if th...

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Is there a role to continue aspirin in patients with myeloproliferative disorders who have never had a thrombotic event that are starting DOAC for stroke prophylaxis with newly diagnosed atrial fibrillation?

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How do you manage a patient with JAK2+ ET without history of thrombosis during pregnancy?

Does history of miscarriage affect your treatment decision?

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How do you differentiate between JAK2 positive ET and PV when peripheral blood shows erythrocytosis and thrombocytosis?

Concordant low EPO level and bone marrow with megakaryocyte hyperplasia

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How would you manage a patient with erythrocytosis of unknown cause in pregnancy?

MPN work-up negative

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Does stopping anagrelide affect fibrosis in patients with ET who develop post-ET myelofibrosis?

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How do you titrate hydroxyurea in the management of myeloproliferative neoplasms?

What parameters/goals/targets do you use? Do you treat PV, ET, and MF differently?

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Would you consider clearing a patient with essential thrombocytosis for a kidney donation?

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How do you manage MPN patients with acquired VWD in the perioperative setting?

Please specify your approach in elective and emergency surgical situations

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How do you manage and/or prevent ruxolitinib withdrawal syndrome?

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What aspirin dose do you favor for thrombotic prophylaxis in myeloproliferative neoplasms?

81 mg vs 162 mg

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Would you continue ruxolitinib in combination with HMA plus venetoclax in myelofibrosis at the time of transformation to AML?

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What are your triggers for BM biopsy in polycythemia vera?

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What workup and initial treatment should be considered for suspected autoimmune myelofibrosis?

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What is the benefit of cytoreduction with Hydroxyurea for patients with newly diagnosed chronic phase CML with a WBC count >100 prior to the initiation of a TKI?

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What platelet threshold, if any, is your goal for cytoreduction in essential thrombocythemia?

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Are there any differences in your approach to therapy for secondary versus primary myelofibrosis?

Secondary MF such as post-PV, for example. Do you apply the same prognostic scoring systems?

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In someone with an asymptomatic JAK2 V617 mutated and BCR/ABL negative MPN with a steadily rising WBC, when do you decide to start cytoreductive therapy and which agent(s) do you recommend?

No evidence of palpable splenomegaly, normal hematocrit and platelet count

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What are your recommendations for management of patients with familial polycythemia due to EPOR mutation during pregnancy?

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How would you manage a patient with essential thrombocythemia and persistent pruritus despite adequate control of CBC with hydroxyurea and aspirin?

ET is JAK2+

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How do you manage recurrent portal vein thromboses in a patient with JAK2+ PV on therapeutic anticoagulation and appropriate cytoreduction?

Recurrent thromboses despite therapeutic enoxaparin. Other hypercoagulable work-up negative.

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Does potential longterm risk for leukemia influence your decision for using hydroxyurea in young patients with myeloproliferative neoplasms?

Would pegylated interferon be preferred?

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Would you consider using HMA in management of polycythemia vera?

i.e. treatment-refractory PV, prior to progression to PMF or AML

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How should one manage a patient with leukocytosis and borderline detectable BCR-ABL without other clinical features of CML such as basophilia or splenomegaly?

e.g. IS < 0.002% by qPCR

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Do you adjust the dose of ruxolitinib in primary myelofibrosis based on hemoglobin?

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Does variable allele frequency (VAF) of JAK mutation affect your clinical decision-making in MPN in any scenario?

Does VAF <1% make you want to do a BM biopsy for confirmation?

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In young patients with essential thrombocythemia and early significant atherosclerotic disease but no thrombotic history, would you consider the addition of cytoreductive therapy?

Would the presence of JAK2 versus CALR versus MPL influence this decision? What if there are other risk factors for cardiovascular disease?

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How would you manage a patient with JAK2-positive PV who is not responding to hydroxyurea or IFN-alpha for cytoreduction?

This is in the setting of a patient who is now on ruxolitinib with rising leukocytosis and thrombocytosis, but cannot be on aspirin due to recent blee...

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What is your experience managing myelosuppression from busulfan, prescribed for PV?

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In patients with high-risk polycythemia vera with a history of thrombosis, should aspirin be used in addition to indefinite anticoagulation and cytoreduction for prevention of recurrent thrombosis?

What if there are no cardiovascular risk factors? Are VKAs preferred or can DOACs be used?

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In patients with thrombocytosis with negative MPN workup from peripheral blood and bone marrow, is there a role for daily 81mg aspirin or other treatment?

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Does cytoreductive therapy in high risk PV reduce thrombotic events because of better normalization of blood counts as compared to phlebotomy, or through some other mechanism?

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In the absence of symptoms would you still treat high risk myelofibrosis if transplant ineligible?

Is leukocytosis and thrombocytosis alone an indication for treatment?

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Would you treat iron deficiency in post PV myelofibrosis if anemia is the predominant concern?

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In a patient with progressive thrombocytosis but negative MPN mutations on peripheral blood, what are your diagnostic and treatment recommendations?

Do you ever recheck JAK2/CALR/MPL/BCR-ABL? Would you recommend a bone marrow biopsy? Is cytoreductive therapy indicated?

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When do you check for EPOR gene mutations in your workup for erythrocytosis, and if found, how do you manage it?

Is there a role for aspirin or hydroxyurea? Do you perform phlebotomy, and if so, what goals?

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How would you approach a young person with JAK2 V617F positive essential thrombocytosis with otherwise low risk features?

Would you get bone marrow biopsy periodically? Would your approach change based off specific age or platelet count?

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What is the role, if any, for next generation sequencing testing in patients with suspected MPN who test negative for commonly identified mutations such as JAK2, CALR, MPL, and BCR-ABL?

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How would you manage symptomatic iron deficiency in patients with PV on frequent phlebotomies?

Is this a reason to start hydrea? Would you give oral iron after adequate control on hydrea?

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Do you recommend anticoagulation for a splenic infarct in patients with myelofibrosis and splenomegaly?

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What is your perioperative approach to holding and restarting hydroxyurea in patients with essential thrombocythemia?

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How would you optimally manage a patient with a MDS/MPN overlap syndrome who has both transfusion-dependent anemia and marked thrombocytosis?

Would this change with someone who has a history of thrombosis (e.g. DVT/PE, MI, CVA)? Would this change with someone who is more fit vs more frail?

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How do you manage testosterone replacement therapy-induced erythrocytosis?

Do you recommend therapeutic phlebotomy to a certain Hct goal? Any strong evidence for thromboembolic risk related to erythrocytosis or if this is mit...

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How often do you monitor thiamine levels in a myelofibrosis patient taking fedratinib?

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Do you consider splenic response as adequate to judge efficacy of JAK2 inhibitors in myeloproliferative disorders?

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How would you manage a patient with PV or ET who is experiencing breakthrough thrombosis?

For example, a patient on cytoreductive therapy and aspirin BID, but suffers an arterial event, or a patient who is already on therapeutic anticoagula...

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How would you manage acquired von Willebrand syndrome secondary to a myeloproliferative neoplasm in a patient who is already on anticoagulation?

Individuals with MPNs may be at higher risk for thrombosis and be placed on antiplatelet therapy or even anticoagulation because of prior thrombosis. ...

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Is there a role for ruxolitinib in essential thrombocythemia?

In patients who are intolerant to hydroxyurea, anagrelide and interferon

1 Answer available