Reader Responses: How would you treat this patient with JAK2-positive essential thrombocythemia?

Here’s how readers responded to a You Make the Call question about anticoagulation in a patient with essential thrombocythemia.


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Based on the information given, as well as the fact that she is of childbearing age, I would consider the use of pegylated interferon, which is safe in pregnancy.

Maria T. DeSancho, MD
New York, NY

I would start ruxolitinib.

Francisco Cuéllar Ambrosi, MD
Medellín, Colombia

Cytoreductive therapy should be offered to patients with PV who develop significant thrombocytopenia as a result of phlebotomy or who have persistent leukocytosis. Persistent Leukocytosis has been shown to increase risk of thrombosis and disease evolution in retrospective studies. Extreme thrombocytosis could cause acquired von Willebrand disease, which can be controlled by cytoreductive therapy.

Yazhini Vallatharasu, MD
Appleton, WI 

The high WBC count is deleterious. I would add hydroxyurea to control it and reduce the need for phlebotomy.

Evan D. Slater, MD
Ventura, CA

I would start cytoreductive therapy.

Elangovan Balakrishnan, MD
Columbia, MO

In a young woman with polycythemia vera who is menstruating, repeated phlebotomies could increase the chance of iron deficiency. The patient presents with high neutrophil and platelet counts that may be associated with vascular events. In my opinion, she should be treated with ropeginterferon alfa-2b.

Arturo Musso, MD
Buenos Aires, Argentina

Although this patient technically does not fit criteria to start cytoreduction (at least here in the U.K., where platelet counts of <1,500 ×109/L are not considered high risk features), I would consider cytoreduction because she has a high JAK2 allele burden, a high WBC count, and a high platelet count, all of which increase her thrombotic risk. She also has the compounding risk of high blood pressure. Tighter control of her hematocrit level, as well as platelet and WBC counts, would all reduce her risk. It simply is not possible to achieve that with intermittent venesection alone. I would, however, use interferon to avoid exposing her to hydroxyurea/hydroxycarbamide at such young age, taking into account that she might want to have children. I assume she is on aspirin, as she should be.

Paolo Gallipoli, MD
London, U.K.

I would start her on pegylated interferon, as she is young and has a very high platelet count.

Ping Han, MD, PhD
Bankstown, Australia

I would start cytoreductive therapy if she is high risk, but would not if she is low risk.

Caroline Jacquy
Mons, Belgium

According to Central European Myeloproliferative Neoplasm Organization guidelines, the patient is very high risk, not low risk. She deserves immediate cytoreductive therapy with ropeginterferon alfa-2b, combined, at first, with hydroxyurea.

Jiří Schwarz, MD, PhD
Prague, Czech Republic

The thrombocytosis is an indication to start cytoreductive therapy.

Mario Tejerina Valle, MD
La Paz, Bolivia

I think we have to consider cytoreductive therapy for low-risk PV if the patient has a high risk of thrombosis in the form of a high leukocyte or platelet count, or if they require frequent visits for phlebotomies and are at risk of ischemic insult, such as young patients with a history of premature coronary artery disease.

Alhasan Ali Bajawi, MD
Mecca, Saudi Arabia

This patient is at higher risk of thromboembolic complications because of her hypertension and leukocytosis. We don’t know if she has been able to keep a hematocrit consistently below 45%. In addition, it is clear she has a very proliferative phenotype. I would recommend cytoreductive therapy in the form of hydroxyurea or alpha interferon, in addition to aspirin, as first lines of therapy are reasonable choices. The goals of therapy should be to keep her hematocrit level below 45%, to bring the WBC count and absolute neutrophil count to a more normal range, and to keep her blood pressure well controlled.

Julio Hajdenberg, MD
Orlando, FL

I would start therapy with peginterferon because of the patient’s young age.

Dubravka Carzavec, MD
Zagreb, Croatia