Myeloproliferative Neoplasms   

Cell counts are normal. Bone marrow shows grade 3 fibrosis. Spleen size continues to enlarge.

qPCR for BCR/ABL was negative. FISH remained positive.

If a patient is unable to tolerate peginterferon, is there any reason to think they may do better with ropeginterferon? 

Is APLS a contraindication to using interferon?

WBC and Hb values are normal, and normal JAK2, CALR, BCR-ABL.

Do you consider "bridging" therapy prior to transplant, and if so, what are your thoughts on the intensity of the chemotherapy?  

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...

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

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

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

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

Does the anagrelide shortage play a role in your decision-making? Do you avoid anagrelide in general?

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...

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. 

How do you use this information in clinical practice?

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...

Does history of miscarriage affect your treatment decision?

Concordant low EPO level and bone marrow with megakaryocyte hyperplasia

MPN work-up negative

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

Please specify your approach in elective and emergency surgical situations

81 mg vs 162 mg

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

No evidence of palpable splenomegaly, normal hematocrit and platelet count

ET is JAK2+

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

Would pegylated interferon be preferred? 

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

e.g. IS < 0.002% by qPCR

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

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

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...

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

Is leukocytosis and thrombocytosis alone an indication for treatment?

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

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

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

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

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?

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

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

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

Would you modify the radiation dose or field in this setting? Would you pre-emptively dose reduce chemotherapy, or modify the dosing of ruxolitinib?

In patients who are intolerant to hydroxyurea, anagrelide and interferon

For instance, does isolated CALR mutated MF change your management?