Service de Dermatologie, CHRU de Brest, Brest, France.
Univ Brest, LIEN, CHRU Brest, Brest, France.
J Eur Acad Dermatol Venereol. 2023 Jun;37(6):1175-1183. doi: 10.1111/jdv.18990. Epub 2023 Mar 9.
Pruritus is a frequent symptom experienced by patients with myeloproliferative neoplasms (MPN). Aquagenic pruritus (AP) is the most common type. The Myeloproliferative Neoplasm-Symptom Assessment Form Total Symptom Score (MPN-SAF TSS) self-report questionnaires were distributed to MPN patients before consultations.
The aim of this study was to assess clinical incidence (phenotypical evolution and response to treatment) of pruritus, especially AP, in MPN patients during their follow-ups.
We collected 1444 questionnaires from 504 patients [54.4% essential thrombocythaemia (ET) patients, 37.7% polycythaemia vera (PV) patients, and 7.9% primary myelofibrosis (PMF) patients].
Pruritus was reported by 49.8% of the patients, including 44.6% of AP patients, regardless of type of MPN or driver mutations. Patients suffering from pruritus were more symptomatic and had a higher rate of evolution into myelofibrosis/acute myeloid leukaemia (19.5% vs. 9.1%, OR = 2.42 [1.39; 4.32], p = 0.0009) than MPN patients without pruritus. Patients with AP had the highest pruritus intensity values (p = 0.008) and a higher rate of evolution (25.9% vs. 14.4%, p = 0.025, OR = 2.07) than patients with non-AP. Disappearance of pruritus was observed in only 16.7% of AP cases, compared to 31.7% of cases with other types of pruritus (p < 0.0001). Ruxolitinib and hydroxyurea were the most effective drugs to reduce AP intensity.
In this study, we demonstrate the global incidence of pruritus across all MPN. Pruritus, especially AP, which is a major constitutional symptom observed in MPN, should be assessed in all MPN patients due to higher symptom burden and higher risk of evolution.
瘙痒是骨髓增殖性肿瘤（MPN）患者常见的症状。水诱发的瘙痒症（AP）是最常见的类型。在就诊前，我们向 MPN 患者分发了骨髓增殖性肿瘤症状评估表总症状评分（MPN-SAF TSS）自报告问卷。
本研究旨在评估 MPN 患者在随访过程中瘙痒症（尤其是 AP）的临床发生率（表型演变和对治疗的反应）。
我们从 504 名患者中收集了 1444 份问卷[54.4%特发性血小板增多症（ET）患者，37.7%真性红细胞增多症（PV）患者和 7.9%原发性骨髓纤维化（PMF）患者]。
49.8%的患者报告有瘙痒症，包括 44.6%的 AP 患者，无论 MPN 类型或驱动突变如何。患有瘙痒症的患者更有症状，且更有可能演变为骨髓纤维化/急性髓系白血病（19.5%比 9.1%，OR=2.42[1.39;4.32]，p=0.0009）。与无瘙痒症的 MPN 患者相比，AP 患者瘙痒症强度值最高（p=0.008），且演变率更高（25.9%比 14.4%，p=0.025，OR=2.07）。仅 16.7%的 AP 病例瘙痒症消失，而其他类型瘙痒症（非 AP）病例为 31.7%（p<0.0001）。鲁索利替尼和羟基脲是降低 AP 强度最有效的药物。
在这项研究中，我们证明了所有 MPN 瘙痒症的总体发生率。瘙痒症，特别是在 MPN 中观察到的主要全身症状的 AP，由于更高的症状负担和更高的演变风险，应在所有 MPN 患者中进行评估。
