Polycythemia vera‐associated pruritus and its management

Saini, Kamal S.; Patnaik, Mrinal M.; Tefferi, Ayalew

doi:10.1111/j.1365-2362.2010.02334.x

Cited by 68 publications

(86 citation statements)

References 60 publications

(149 reference statements)

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“…Pruritus, typically acquagenic, is the most frequent and disabling complaint of patients with PV, and is reported in up to 70% of cases. 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.…”

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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“…Side effects (autoimmune disorders, flu-like manifestations, depression, heart, and ocular disease) lead to permanent discontinuation in 20% to 40% on conventional and 20% to 25% on pegylated interferon. However, both hydroxyurea and interferon are poorly effective in relieving symptoms, although the latter may help some patients with severe itching 68 ; also, the effects on splenomegaly are usually modest and transient. I do not consider thrombocytosis (unless extreme, .1000 3 10 9 /L), in otherwise asymptomatic lowrisk patients, a good reason to start cytotoxic therapy, nor do I adjust the dose of cytotoxic drugs in otherwise well-controlled high-risk patients only to normalize platelet count.…”

Section: Therapy Goals and Therapy Optionsmentioning

confidence: 99%

How I treat polycythemia vera

Vannucchi

2014

Blood

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Polycythemia vera (PV) is a chronic myeloproliferative neoplasm associated with JAK2 mutations (V617F or exon 12) in almost all cases. The World Health Organization has defined the criteria for diagnosis, but it is still unclear which parameter (hemoglobin or hematocrit) is the most reliable for demonstrating increased red cell volume and for monitoring response to therapy; also, the role of bone marrow biopsy is being revisited. PV is associated with reduced survival because of cardiovascular complications and progression to post-PV myelofibrosis or leukemia. Criteria for risk-adapted treatment rely on the likelihood of thrombosis. Controlled trials have demonstrated that incidence of cardiovascular events is reduced by sustained control of hematocrit with phlebotomies (low-risk patients) and/or cytotoxic agents (high-risk patients) and antiplatelet therapy with aspirin. Hydroxyurea and interferon may be used as first-line treatments, whereas busulfan is reserved for patients that are refractory or resistant to first-line agents. However, there is no evidence that therapy improves survival, and the significance of reduction of JAK2 mutated allele burden produced by interferon is unknown. PV is also associated with a plethora of symptoms that are poorly controlled by conventional therapy. This article summarizes my approach to the management of PV in daily clinical practice.

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“…It is characterized by strong itching, stinging, tingling, or burning sensations following contact with water without visible changes of the skin [2,3]. Since it may strongly impair quality of life in affected patients and is responsible for much of the chronic suffering in PV [4], a better understanding for better treatment is mandatory [5].…”

Section: Introductionmentioning

confidence: 99%

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

2013

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Aquagenic pruritus (AP) is a symptom typical for polycythemia vera, but very little is known about its exact frequency, characteristics, influence on quality of life, and proper treatment. Therefore, we investigated these aspects in a large cohort of German patients with polycythemia vera using a patient directed questionnaire. Our analysis revealed that 301 of 441 analyzed patients suffered from AP. In 64.8%, AP occurred on average 2.9 years prior to diagnosis of polycythemia vera. Only in 15.4% did this lead to a hematological investigation. AP occurs primarily on the trunk and proximal parts of the extremities. Most patients complain about itching (71.8%), the remainder about tickling, stinging, or burning sensations. Forty‐four patients (14.6%) classified the pruritus as “unbearable.” Patients with AP reported reduced global health status and higher fatigue, pain, and dyspnea. Only 24% of patients received pruritus specific treatment for pruritus consisting mostly of histamine antagonists, which ameliorated symptoms in about half of the patients. In 5.6% of patients, polycythemia vera directed therapy (phlebotomy/cytoreduction) resolved the symptoms. In summary, AP is a serious symptom in patients with polycythemia vera, which until recently was difficult to treat. The advent of the novel JAK2 inhibitors, however, may open new ways for therapy. Am. J. Hematol. 88:665–669, 2013. © 2013 Wiley Periodicals, Inc.

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Polycythemia vera‐associated pruritus and its management

Abstract: Background Pruritus is a defining feature of polycythemia vera (PV) and is seen in approximately 40% of patients. In most cases, the pruritus is characteristically triggered by contact with water (aquagenic) at any temperature.

Cited by 68 publications

References 60 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

How I treat polycythemia vera

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

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