Ruxolitinib (a JAK2 inhibitor) as an emerging therapy for refractory pruritis in a patient with low-risk polycythemia vera: A case report

Abstract

Introduction: Polycythemia vera (PV) is a Philadelphia-negative myeloproliferative neoplasm (MPN) characterized by the overproduction of red blood cells. The presence of JAK2 mutation is detected in up to 99% of patients with PV. Pruritis is commonly encountered in patients with PV and is considered the most troublesome symptom. Multiple treatment modalities are used for treatment; however, their efficacy is variable. Sometimes, pruritis will not improve even by the use of combined therapies. Recently, Ruxolitinib (a JAK2 inhibitor) has been shown to be very effective, especially in patients with refractory pruritis in the setting of other treatment modalities failure.

Patient concern: We describe a 55-year-old male with history of low risk PV presented with severe itching despite using different therapies, including phlebotomy and hydroxyurea. His laboratory results on presentation were significant for red blood cells (RBC) of 8.2 × 106/uL (normal = 4.5-5.5), hematocrit (Hct) of 52.8% (normal = 40-50%), platelet count of 519 × 103/uL (normal = 150-400), white blood cells count of 12.3 × 103/uL (normal = 4-10), and basophils count of 0.22 × 103/uL (normal < 0.1).

Diagnosis: PV related refractory pruritis.

Intervention: Pruritis improved dramatically after starting Ruxolitinib therapy with an improvement of hematological parameters (both hematocrit and platelet count).

Conclusion: Different treatment modalities have shown to be beneficial in treating PV-related pruritis, but the clinical outcomes are highly variable. This case report aims to shed light on Ruxolitinib as an emerging therapy for the treatment of refractory cases of PV-related pruritis.