Aquagenic pruritus in polycythemia vera: Characteristics and influence on quality of life in 441 patients

Siegel, Fabian; Tauscher, Jan; Petrides, Petro E.

doi:10.1002/ajh.23474

Cited by 94 publications

(104 citation statements)

References 26 publications

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“…62 In extreme situations (approx. 15%) 63 it causes severe disruption of the individual's lifestyle, inducing sleep disturbances, depression, and impaired working capabilities and social relationships. In a recent landmark study into MPN in the United States that interviewed 380 PV patients undergoing treatment, fatigue and itching were identified by 33% and 9% of the respondents as the symptom they most urgently wanted to resolve.…”

Section: Recognizing the Burden Associated With Diseasementioning

confidence: 99%

From leeches to personalized medicine: evolving concepts in the management of polycythemia vera

Vannucchi

2016

Haematologica

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© Ferrata Storti Foundationing to the level of knowledge we have now. Table 1 lists some of these landmark studies; due to space constraints, I will not be able to address all of them in detail. Evolving concepts in diagnosis Making a diagnosis of polycythemia veraThe World Health Organization (WHO) recently released a revised classification of MPN in which important changes to the 2008 version were introduced (Table 2). 8 In the 2008 version, the most compelling innovation had been the introduction of JAK2V617F and "similar" mutations (involving JAK2 exon 12 in 3%-4% of patients) as major diagnostic criteria.3-6 Although JAK2V617F mutation is associated with PV in more than 95% of cases, it does not represent a clear diagnosis since it is found also in 50%-60% of ET and PMF. However, the use of JAK2V617F as a marker of clonal myeloproliferation greatly facilitates the distinction of PV from reactive or congenital erythrocytosis.Considering that isotope-based assays for measuring red cell mass (RCM) and plasma volume are not routinely available even in most tertiary centers, the 2008 WHO classification listed a hemoglobin level more than 185 g/L and 165 g/L in men and women, respectively, as a strong surrogate marker of absolute increase of RCM. Since some PV patients do not fulfill such high levels, other criteria were added to facilitate diagnosis, including: 1) hemoglobin or hematocrit level that is more than 99 th percentile of reference range for age, sex, or altitude of residence; 2) an RCM that is more than 25% above mean normal predicted value;3) a hemoglobin level more than 170 g/L and 150 g/L in men and women, associated with a sustained increase of 20 g/L from baseline not attributable to correction of iron deficiency. According to the pragmatic British standards, hematocrit more than 52% in males and more than 48% in females, or an RCM more than 25% above predicted value, are sufficient to establish a diagnosis of PV if JAK2 mutation is present. 9 However, a reassessment of how far the WHO criteria can be applied in a real-life setting raised the issue of JAK2V617F mutated patients with only a borderline increase in hemoglobin. It was shown that BM morphology, according to WHO guidelines, accurately reflected a condition of increased RCM, since all patients with increased RCM also had a BM morphology consistent with PV.

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Section: Recognizing the Burden Associated With Diseasementioning

confidence: 99%

From leeches to personalized medicine: evolving concepts in the management of polycythemia vera

Vannucchi

2016

Haematologica

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“…Other common aspects of the PV disease state, including blood hyperviscosity (Barbui et al , 2013) and splenomegaly (Mesa et al , 2007), may also play a role in the symptom profile of some patients. The overall PV‐related symptom burden, as measured by the MPN Symptom Assessment Form total symptom score (MPN‐SAF TSS), as well as the severity of individual symptoms, including fatigue (Emanuel et al , 2012; Abelsson et al , 2013) and itching (Siegel et al , 2013), have been associated with reduced quality of life in patients with PV.…”

mentioning

confidence: 99%

The efficacy and safety of continued hydroxycarbamide therapy versus switching to ruxolitinib in patients with polycythaemia vera: a randomized, double‐blind, double‐dummy, symptom study (RELIEF)

et al. 2016

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SummaryThe randomized, double‐blind, double‐dummy, phase 3b RELIEF trial evaluated polycythaemia vera (PV)‐related symptoms in patients who were well controlled with a stable dose of hydroxycarbamide (also termed hydroxyurea) but reported PV‐related symptoms. Patients were randomized 1:1 to ruxolitinib 10 mg BID (n = 54) or hydroxycarbamide (prerandomization dose/schedule; n = 56); crossover to ruxolitinib was permitted after Week 16. The primary endpoint, ≥50% improvement from baseline in myeloproliferative neoplasm ‐symptom assessment form total symptom score cytokine symptom cluster (TSS‐C; sum of tiredness, itching, muscle aches, night sweats, and sweats while awake) at Week 16, was achieved by 43·4% vs. 29·6% of ruxolitinib‐ and hydroxycarbamide‐treated patients, respectively (odds ratio, 1·82; 95% confidence interval, 0·82–4·04; P = 0·139). The primary endpoint was achieved by 34% of a subgroup who maintained their hydroxycarbamide dose from baseline to Weeks 13–16. In a post hoc analysis, the primary endpoint was achieved by more patients with stable screening‐to‐baseline TSS‐C scores (ratio ≤ 2) receiving ruxolitinib than hydroxycarbamide (47·4% vs. 25·0%; P = 0·0346). Ruxolitinib treatment after unblinding was associated with continued symptom score improvements. Adverse events were primarily grades 1/2 with no unexpected safety signals. Ruxolitinib was associated with a nonsignificant trend towards improved PV‐related symptoms versus hydroxycarbamide, although an unexpectedly large proportion of patients who maintained their hydroxycarbamide dose reported symptom improvement.

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“…− A cool and lukewarm bath should be recommended in order to relieve itching in the patient [26,52].…”

Section: Deterioration Of Comfort Itchingmentioning

confidence: 99%

Nursing Care in Polycythemia Vera: A Review Article

Özkaraman¹,

Babadağ²,

Hi³

2017

J Pat Care

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IntroductionPolycythemia vera (PV) is a chronic myeloproliferative neoplasm, which is characterised with the clonal proliferation of the erythroid, myeloid and megakaryocytic series and which is a threat to life [1,2]. The foundation is prepared for the formation of blood clots in all of the tissues and organs, together with an increase in the viscosity and volume of blood in particular, and many systems such as the central nervous system and the cardiovascular and gastrointestinal systems are affected [1,3]. While PV, which increases with age, is seen in a rate of 10.9 out of every one million people in America [4], it has not been possible to reach data on the incidence of the illness in Turkey. According to the diagnosis criteria of the World Health Organisation, 2 major and 1 minor or 1 major and 2 minor criterion need to be present at the same time for a diagnosis of PV. The major criteria are haemoglobin levels in men being >18.5 g/dl and >16.5 g/dl in women, or the presence of the other findings of increased masses; and the positivity of other functionally similar mutations, such as the JAK2 and V61F7 or JAK2 Exon 12 mutation. The minor criteria, on the other hand, are a bone marrow biopsy displaying hyper-cellularity based on age, lower than normal serum erythropoietin levels and the formation of in vitro endogenous erythroid colonies [5].The symptoms in polycythemia patients are generally asymptomatic, while generally being headaches due to hyper-viscosity and hypervolemia, as well as nose bleeds, tinnitus, vertigo, vision disorders, dyspnoea, angina, a florid appearance and a burning sensation in the hands and feet. Additionally, symptoms of petechia, purpura, ecchymosis, splenomegaly, hepatomegaly, peptic ulcers and hyperuricaemia are seen [6,7]. Cardiovascular risk factors (hypertension, hypercholesterolemia, diabetes, smoking) increase the risk of complications in advanced ages in particular [5,8] AbstractBackground: Polycythemia vera (PV) is a chronic myeloproliferative neoplasm, which is a threat to life. Teamwork is needed during the process of treatment and care of patients suffering from PV, which is a chronic illness. Especially, important roles fall to the nurses within the team.

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Aquagenic pruritus in polycythemia vera: Characteristics and influence on quality of life in 441 patients

Cited by 94 publications

References 26 publications

From leeches to personalized medicine: evolving concepts in the management of polycythemia vera

From leeches to personalized medicine: evolving concepts in the management of polycythemia vera

The efficacy and safety of continued hydroxycarbamide therapy versus switching to ruxolitinib in patients with polycythaemia vera: a randomized, double‐blind, double‐dummy, symptom study (RELIEF)

Nursing Care in Polycythemia Vera: A Review Article

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