Differences between aquagenic and non‐aquagenic pruritus in myeloproliferative neoplasms: An observational study of 500 patients

Abstract

Myeloproliferative neoplasms (MPN) are chronic disorders of myeloid lineages. The three classical MPN are polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF). MPN are clonal disorders due to the acquisition of driver mutations, JAK2, calreticulin (CALR) and MPL, that affect proliferation signalling pathways. Proportion and repartition are different between MPN, but JAK2V617F is the most frequent mutation. These neoplasms expose patients to high risk of complications such as thrombosis, haemorrhage and phenotypical evolution [secondary myelofibrosis (MF), accelerated phase (AcPh) or acute myeloid leukaemia (AML)]. The risk of evolution depends on the MPN subtype

Reply to ‘Omalizumab for the management of refractory aquagenic pruritus’

“…We agree with the authors on their statements about standard antihistamine drugs not having a resolving effect on AP and there is currently no standard cure for AP, and treatment options are limited. [2][3][4] We disagree on the other hand about the role of omalizumab for AP; the authors state that 2 cases have been published in the literature prior to their experience in which it would appear that omalizumab may play a role in AP, in fact, this statement is incorrect as there is only one case of treatment with omalizumab for AP, the other case cited by the authors' concerns of a case of aquagenic urticaria which represents a disease entity distinct from AP. 5,6 Our experience is the first dealing with a case series of patients with AP where instead omalizumab was discontinued due to ineffectiveness therefore we believe that these single experiences described by the authors although describing successfully treated cases need further detailed data to be confirmed.…”

Omalizumab for the management of refractory aquagenic pruritus

“…1 Its pathogenesis is not well understood, but it may involve the activation of nociceptive nerve fibres in response to osmotic or mechanical stress induced by water exposure. 2 This type of pruritus is the most common and significant form of pruritus seen in myeloproliferative neoplasms (MPN) and can have a direct and indirect impact on the quality of life (QoL) of those affected. 2,3 AP is a condition where standard antipruritic medication, such as antihistamines, does not work well, indicating that there may be other causes involved.…”

“…2 This type of pruritus is the most common and significant form of pruritus seen in myeloproliferative neoplasms (MPN) and can have a direct and indirect impact on the quality of life (QoL) of those affected. 2,3 AP is a condition where standard antipruritic medication, such as antihistamines, does not work well, indicating that there may be other causes involved. We describe a case of AP that did not respond to traditional treatments but was effectively treated with the anti-IgE antibody drug omalizumab.…”

Aquagenic pruritus indicates increased risks in patients with myeloproliferative neoplasms

“…In this issue of JEADV , Christelle Le Gall‐Ianotto et al presented their data from a retrospective study in which they set out to determine the clinical incidence of pruritus and especially AP in patients with myeloproliferative diseases. They also tracked the evolution of these symptoms over time and their response to treatment by using a specific questionnaire before and during the patient follow‐up 7 …”

“…They also tracked the evolution of these symptoms over time and their response to treatment by using a specific questionnaire before and during the patient follow-up. 7 They collected 1444 Myeoloproliferative Neopolasm-Symptom Assessment Form Total Symptom (MPN-SAF TSS) self-report questionnaires from 504 MPN patients with a median time between the first and last questionnaire of 4.1 years. Among these 504 patients, 54.4% had essential thrombocythemia (ET), 37.7% had polycythemia vera (PV), and 7.9% had primary myelofibrosis (PMF).…”