Polycythemia vera‐associated pruritus and its management

European Journal of Clinical Investigation - Tập 40 Số 9 - Trang 828-834 - 2010
Kamal S. Saini1, Mrinal M. Patnaik2, Ayalew Tefferi2
1Department of Medical Oncology, Institute Jules Bordet, Brussels, BELGIUM
2Division of Hematology Mayo Clínic Rochester MN USA

Tóm tắt

Eur J Clin Invest 2010; 40 (9): 828–834AbstractBackground  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.Materials and methods  A detailed MEDLINE search for all English language articles related to PV, PV‐associated pruritus and aquagenic pruritus that were published from 1965 till date was carried out.Results  Many different treatment options have been tried over the past several decades, including antihistamines, antidepressants, interferon alpha, phlebotomy, phototherapy, iron supplements and myelosuppressive medications, all demonstrating mixed results. Recently, agents that target JAK2 and mammalian target of rapamycin (mTOR) have shown impressive clinical benefit.Conclusion  PV‐associated pruritus is a major cause of morbidity amongst patients with PV. Antidepressant medications interfering with serotonin uptake are somewhat efficacious. Cytoreductive therapy is reserved for refractory cases. Targeted therapy with JAK2 and mTOR inhibitors offers renewed hope.

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Tài liệu tham khảo

10.1056/NEJMoa051113

10.1016/S0140-6736(05)71142-9

Vannucchi AM, 2007, Clinical profile of homozygous JAK2 617V>F mutation in patients with polycythemia vera or essential thrombocythemia, Blood, 3, 840, 10.1182/blood-2006-12-064287

Brumpt L, 1952, [Pruritus in polycythemia.], Presse Med, 66, 1397

10.1046/j.1365-2141.2001.03161.x

Arroyo H, 1971, [The bath sign], Presse Med, 41, 1814

10.1016/S0190-9622(85)70149-1

10.1111/j.1600-0609.1975.tb01054.x

10.1002/ajh.21156

10.1111/j.1365-2133.1987.tb05787.x

10.1002/ajh.2830430419

10.1002/cncr.22365

10.1182/blood.V28.6.795.795

10.1016/S0140-6736(88)92632-3

10.1016/S0140-6736(88)92254-4

Kligman AM, 1986, Water‐induced itching without cutaneous signs. Aquagenic pruritus, Arch Dermatol, 122, 183, 10.1001/archderm.1986.01660140073021

Means R, 2009, Wintrobe’s Clinical Hematology, 2031

10.1159/000247223

10.1016/0140-6736(91)92206-H

10.3109/10428190009148845

10.1136/bmj.282.6281.2008

10.1159/000247891

10.1111/j.1365-4362.1986.tb00863.x

10.1016/S0190-9622(88)70110-3

10.1111/j.1468-3083.2004.01023.x

Diamond JH, 1966, Urticaria pigmentosa complicated by polycythemia vera. Report of a case, Blood, 27, 253, 10.1182/blood.V27.2.253.253

10.1111/j.1600-0560.2007.00927.x

Bircher AJ, 1988, Aquagenic pruritus. Water‐induced activation of acetylcholinesterase, Arch Dermatol, 124, 84, 10.1001/archderm.1988.01670010048020

Ishii T, 2009, Pivotal role of mast cells in pruritogenesis in patients with myeloproliferative disorders, Blood, 23, 5942, 10.1182/blood-2008-09-179416

10.1038/leu.2009.85

10.1002/cncr.21645

10.3324/haematol.2009.007047

10.7326/0003-4819-123-9-199511010-00003

10.1200/JCO.2005.07.062

Wasserman LR, 1981, Influence of therapy on causes of death in polycythemia vera, Trans Assoc Am Physicians, 94, 30

10.1001/archinte.142.2.241

10.1038/leu.2008.280

10.7326/0003-4819-119-11-199312010-00006

10.1002/cncr.22026

10.1007/s002770050563

10.1111/j.1365-2141.1995.tb03306.x

10.1182/blood-2006-03-009860

10.1002/1097-0142(19860215)57:4<718::AID-CNCR2820570406>3.0.CO;2-P

10.1002/ajh.2830410120

10.1002/(SICI)1096-8652(199602)51:2<112::AID-AJH3>3.0.CO;2-0

10.1080/095466301317085408

10.1007/s002770050207

10.1046/j.1365-2133.2002.04983.x

10.1111/j.1365-2133.1993.tb03520.x

10.1182/blood.V99.7.2627

Verstovsek S, 2009, A Phase 2 Study of INCB018424, an oral, selective JAK1/JAK2 inhibitor, in patients with advanced polycythemia vera (PV) and essential thrombocythemia (ET) refractory to hydroxyurea, Blood, 114, 631, 10.1182/blood.V114.22.311.311

Pardanani A, 2009, A Phase I evaluation of TG101348, a selective JAK2 inhibitor, in myelofibrosis: clinical response is accompanied by significant reduction in JAK2V617F allele burden ASH, Blood, 114, 755, 10.1182/blood.V114.22.755.755

Vannucchi AM, 2009, RAD001, an inhibitor of mTOR, shows clinical activity in a phase I/II study in patients with primary myelofibrosis (PMF) and post polycythemia vera/essential thrombocythemia myelofibrosis (PPV/PET MF), Blood, 114, 10.1182/blood.V114.22.307.307

10.1038/sj.leu.2401045

10.1111/j.1365-2141.1987.00285.x

10.1002/(SICI)1096-8652(199709)56:1<59::AID-AJH12>3.0.CO;2-Y

Verstovsek SKH, 2009, The JAK inhibitor, INCB018424, demonstrates durable and marked clinical responses in primary myelofibrosis (PMF) and post‐polycythemia/essential thrombocythemia myelofibrosis (Post PV/ETMF), Blood, 114

Pardanani AD, 2009, A phase I evaluation of TG101348, a selective JAK2 inhibitor, in myelofibrosis: clinical response is accompanied by significant reduction in JAK2V617F allele burden, Blood, 114, 10.1182/blood.V114.22.755.755