Pruritus in primary myelofibrosis: management options in the era of JAK inhibitors

Vaa, Brianna E.; Tefferi, Ayalew; Gangat, Naseema; Pardanani, Animesh; Lasho, Terra L.; Finke, Christy; Wolanskyj, Alexandra P.

doi:10.1007/s00277-016-2674-2

Cited by 13 publications

(7 citation statements)

References 44 publications

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“…The latter observation was confirmed by others as well. Treatment options for PV-associated pruritus include antihistamines 64 , selective serotonin reuptake inhibitors (SSRIs) 65 , danazol 66 , IFN-α 67 , narrow-band ultraviolet B phototherapy 68 , photochemotherapy with psoralen and ultraviolet A light (PUVA) 69 , and JAK2 70 or mTOR inhibitors 71 . Amongst these, we recommend initial therapy with antihistamines and SSRIs, followed by IFN-α therapy for more severe and refractory cases, and reserve treatment with JAK2 inhibitors for high-risk patients with IFN-α-resistant pruritus.…”

Section: Risk-adapted Treatment Algorithm In Polycythemia Veramentioning

confidence: 99%

Polycythemia vera treatment algorithm 2018

Tefferi

Vannucchi

Barbui

2018

Blood Cancer Journal

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Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera (PV), with an estimated median survival of 24 years, in patients younger than age 60 years old. Currently available drugs for PV have not been shown to prolong survival or alter the natural history of the disease and are instead indicated primarily for prevention of thrombosis. Unfortunately, study endpoints that are being utilized in currently ongoing clinical trials in PV do not necessarily target clinically or biologically relevant outcomes, such as thrombosis, survival, or morphologic remission, and are instead focused on components of disease palliation. Even more discouraging has been the lack of critical appraisal from “opinion leaders”, on the added value of newly approved drugs. Keeping these issues in mind, at present, we continue to advocate conservative management in low-risk PV (phlebotomy combined with once- or twice-daily aspirin therapy) and include cytoreductive therapy in “high-risk” patients; in the latter regard, our first, second, and third line drugs of choice are hydroxyurea, pegylated interferon-α and busulfan, respectively. In addition, it is reasonable to consider JAK2 inhibitor therapy, in the presence of protracted pruritus or markedly enlarged splenomegaly shown to be refractory to the aforementioned drugs.

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Section: Risk-adapted Treatment Algorithm In Polycythemia Veramentioning

confidence: 99%

Polycythemia vera treatment algorithm 2018

Tefferi

Vannucchi

Barbui

2018

Blood Cancer Journal

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“…Of the total number of patients with myelofibrosis-related pruritus, about 50% have aquagenic pruritus. 34 , 37 , 39 , 40 …”

Section: Nonspecific Skin Lesions Related To Systemic Hematologic Mal...mentioning

confidence: 99%

“…Phototherapy is also effective in many cases. 34 , 35 , 37 , 39 Aspirin has shown moderate results in cases of PV, and recent studies have encouraged the use of JAK and mTOR inhibitors in pruritus related to PV and primary myelofibrosis. 40 Corticosteroids can be used to control pruritus in intractable lymphomas.…”

Section: Nonspecific Skin Lesions Related To Systemic Hematologic Mal...mentioning

confidence: 99%

Dermatological manifestations of hematologic neoplasms. Part II: nonspecific skin lesions/paraneoplastic diseases

Souza¹,

Amorim²,

Sousa³

et al. 2023

Anais Brasileiros de Dermatologia

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“…More favorable responses have been reported with use of selective serotonin reuptake inhibitors [164] and narrow-band ultraviolet B phototherapy [165]. In high-risk disease setting, both JAK2 inhibitors [166,167] and IFN-a [168] have shown therapeutic activity, which is not shared by HU.…”

Section: Management Of Pruritusmentioning

confidence: 99%

Polycythemia vera: historical oversights, diagnostic details, and therapeutic views

2021

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Polycythemia vera (PV) is a relatively indolent myeloid neoplasm with median survival that exceeds 35 years in young patients, but its natural history might be interrupted by thrombotic, fibrotic, or leukemic events, with respective 20-year rates of 26%, 16%, and 4%. Current treatment strategies in PV have not been shown to prolong survival or lessen the risk of leukemic or fibrotic progression and instead are directed at preventing thrombotic complications. In the latter regard, two risk categories are considered: high (age >60 years or thrombosis history) and low (absence of both risk factors). All patients require phlebotomy to keep hematocrit below 45% and once-daily low-dose aspirin, in the absence of contraindications. Cytoreductive therapy is recommended for high-risk or symptomatic low-risk disease; our first-line drug of choice in this regard is hydroxyurea but we consider pegylated interferon as an alternative in certain situations, including in young women of reproductive age, in patients manifesting intolerance or resistance to hydroxyurea therapy, and in situations where treatment is indicated for curbing phlebotomy requirement rather than preventing thrombosis. Additional treatment options include busulfan and ruxolitinib; the former is preferred in older patients and the latter in the presence of symptoms reminiscent of post-PV myelofibrosis or protracted pruritus. Our drug choices reflect our appreciation for long-term track record of safety, evidence for reduction of thrombosis risk, and broader suppression of myeloproliferation. Controlled studies are needed to clarify the added value of twice- vs once-daily aspirin dosing and direct oral anticoagulants. In this invited review, we discuss our current approach to diagnosis, prognostication, and treatment of PV in general, as well as during specific situations, including pregnancy and splanchnic vein thrombosis.

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Pruritus in primary myelofibrosis: management options in the era of JAK inhibitors

Cited by 13 publications

References 44 publications

Polycythemia vera treatment algorithm 2018

Polycythemia vera treatment algorithm 2018

Dermatological manifestations of hematologic neoplasms. Part II: nonspecific skin lesions/paraneoplastic diseases

Polycythemia vera: historical oversights, diagnostic details, and therapeutic views

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