Department of Dermatology, University Hospital of Brest, Brest, France.
Laboratory of Interactions Neurons-Keratinocytes, University of Brest, Brest, France.
J Thromb Haemost. 2019 Nov;17(11):1950-1955. doi: 10.1111/jth.14588. Epub 2019 Sep 8.
Thromboses and phenotypic evolutions (leukemia, myelofibrosis) are the most frequent complications in polycythemia vera (PV) and essential thrombocythemia (ET). Aquagenic pruritus (AP) is not only PV symptom, but is also present in ET. The presence of pruritus in PV is associated with a lower risk of arterial thrombosis.
To date, no equivalent study has been done to analyse the impact of AP for ET patients.
MATERIALS & METHODS: We used the data from our cohort of patients with myeloproliferative neoplasms seen in our institution (OBENE database, NCT02897297). We collect information at diagnosis, presence or not of AP and all types of complications during their follow-up. To avoid masked PV, all JAK2 positive cases were tested isotopic red mass cell if appropriate.
Among 396 ET patients, presence of AP was found in 42 (10.6%). ET patients with AP were more proliferative, more symptomatic at diagnosis and more difficult to treat. Furthermore, they presented increased risk of thromboses (30.9 versus 17%, P = .03; OR = 2.2 [1.01;4.66]) and phenotypic evolutions (33.3 versus 13.3%, P = .0007; OR = 3.2 [1.44;6.77]), during follow-up.
Aquagenic pruritus is classically associated to PV. But we confirmed here that AP is also present in ET and characterizes patients with higher risk of morbidity (thrombotic events and phenotypic evolutions).
The systematic determination of the presence of AP in ET patients should permit us to better identify these high-risk patients for better management and follow-up.
血栓形成和表型演变（白血病、骨髓纤维化）是真性红细胞增多症（PV）和原发性血小板增多症（ET）最常见的并发症。水诱发的瘙痒（AP）不仅是 PV 的症状，也存在于 ET 中。PV 患者瘙痒的存在与动脉血栓形成的风险降低相关。
迄今为止，尚未有研究分析 AP 对 ET 患者的影响。
我们使用了我们机构的骨髓增殖性肿瘤患者队列（OBENE 数据库，NCT02897297）的数据。我们在诊断时收集信息，存在或不存在 AP 以及在随访期间所有类型的并发症。为了避免掩盖 PV，所有 JAK2 阳性病例均进行同位素红细胞质量检测。
在 396 例 ET 患者中，有 42 例（10.6%）存在 AP。有 AP 的 ET 患者更具增生性，在诊断时更具症状且更难以治疗。此外，他们在随访期间血栓形成（30.9%对 17%，P =.03；OR = 2.2 [1.01；4.66]）和表型演变（33.3%对 13.3%，P =.0007；OR = 3.2 [1.44；6.77]）的风险增加。
水诱发的瘙痒通常与 PV 相关。但我们在此证实，AP 也存在于 ET 中，并可用于识别具有更高发病风险（血栓形成事件和表型演变）的患者。
在 ET 患者中系统确定 AP 的存在，应使我们能够更好地识别这些高危患者，以便进行更好的管理和随访。
