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Case Reports
. 2016 Aug 4;375(5):494-6.
doi: 10.1056/NEJMc1600337.

Clinical Improvement with JAK2 Inhibition in Chuvash Polycythemia

Affiliations
Case Reports

Clinical Improvement with JAK2 Inhibition in Chuvash Polycythemia

Amy W Zhou et al. N Engl J Med. .
No abstract available

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Figures

Figure 1
Figure 1. Patient Characteristics at Baseline and Clinical Response to Ruxolitinib in Three Patients with Chuvash Polycythemia
Panel A shows laboratory values, phlebotomy requirement, and symptoms for all three patients before treatment and during ruxolitinib therapy. ND denotes not determined. Panel B shows the effects of ruxolitinib on platelet-count oscillations in Patient CP-01 and the effects of ruxolitinib on hematocrit levels and phlebotomy requirements in Patients CP-02 and CP-03.

References

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    1. Ang SO, Chen H, Hirota K, et al. Disruption of oxygen homeostasis underlies congenital Chuvash polycythemia. Nat Genet. 2002;32:614–621. - PubMed
    1. Sergueeva AI, Miasnikova GY, Polyakova LA, Nouraie M, Prchal JT, Gordeuk VR. Complications in children and adolescents with Chuvash polycythemia. Blood. 2015;125:414–415. - PMC - PubMed
    1. Gordeuk VR, Prchal JT. Vascular complications in Chuvash polycythemia. Semin Thromb Hemost. 2006;32:289–294. - PubMed
    1. Russell RC, Sufan RI, Zhou B, et al. Loss of JAK2 regulation via a heterodimeric VHL-SOCS1 E3 ubiquitin ligase underlies Chuvash polycythemia. Nat Med. 2011;17:845–853. - PMC - PubMed

Supplementary concepts