Pruritus May Mark Severe Symptomology in Myeloproliferative Neoplasms

April 11, 2023

Jessica Nye, PhD

In myeloproliferative neoplasms (MPNs), patients reporting pruritus had more symptoms, greater symptoms severity, and were more likely to experience disease evolution, according to results of a questionnaire-based study published in Journal of the European Academy of Dermatology and Venerology.

Researchers from Brest University Hospital in France sourced data for this study from the Observatoire Brestois des Néoplasies Myéloprolifératives (OBENE) database. Patients (N=504) with MPNs were given a questionnaire about symptoms and quality of life prior to consultations between 2015 and 2020. Symptoms and severity were self-reported using the Visual Analogue Scale score.

Participants had a median age of 68.6 years, 56.5% were women, 54.4% had essential thrombocythemia (ET), 37.7% had polycythemia vera (PV), and 7.9% primary myelofibrosis (PMF). Most participants (77.4%) had Janus activated kinase 2 (JAK2)-mutated disease. The most common treatments were hydroxyurea (35.1%), pegylated-interferon treatment (8.9%), and anagrelide (7.1%).

Overall, 49.8% of patients reported pruritus. Study participants with pruritus were older (P =.01), more had PV and fewer had ET or PMF (P =.004), more had JAK2-mutated disease (P <.0001), and fewer abstained from treatment (P =.014) compared with those in the nonpruritus group.

We clearly showed the importance of identifying patients with pruritus, who are more symptomatic and at the highest risk of phenotypic evolutions.

Participants with pruritus were more likely to report abdominal discomfort (odds ratio [OR], 3.8), perspiration (OR, 3.29), fatigue (OR, 2.68), concentration problems (OR, 2.65), bone pain (OR, 2.31), early satiety (OR, 2.27), and inactivity (OR, 2.06) and all symptoms were reported to be more intense (all P ≤.00007) compared with patients without pruritus.

Participants with pruritus were more likely to experience disease evolution overall (19.5% vs 9.1%; P =.0009) and specifically to myelofibrosis (13.9% vs 6.3%; P =.0049) and accelerated phase or acute myeloid leukemia (7.2% vs 3.2%; P =.046) compared with those without pruritus, respectively.

Among those in the pruritus group, nearly one-half (44.6%) had aquagenic pruritus (AP). The patients with AP were more likely to be men (P =.0015), more had PV and fewer had ET or PMP (P <.0001), more had JAK2-mutated disease (P =.005), and fewer abstained from treatment (P =.001) compared with those in the non-AP group with pruritus.

Participants with AP did not report more symptoms or more intense symptoms than the non-AP group. However, more patients with AP had disease evolution (25.9% vs 14.4%; P =.025), specifically to myelofibrosis (21.4% vs 7.9%; P =.003) compared with the non-AP group, respectively.

These findings may have been biased, as patients completed the questionnaires prior to consultation with the clinician.

Researchers conclude, “We clearly showed the importance of identifying patients with pruritus, who are more symptomatic and at the highest risk of phenotypic evolutions. Furthermore, we found differences in patients with AP compared to those with non-AP. […] Therefore, clinicians must go beyond simply determining the presence or absence of pruritus to determine whether patients are experiencing AP.”

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.References:

Gall-Ianotto CL, Ficheux A-S, Lippert E, et al. Differences between aquagenic and non-aquagenic pruritus in myeloproliferative neoplasms: an observational study of 500 patients. J Eur Acad Dermatol Venereol. Published online February 21, 2023. doi:10.1111/jdv.18990

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