How I treat myelofibrosis

被引:116
|
作者
Cervantes, Francisco [1 ]
机构
[1] Univ Barcelona, Hosp Clin, Dept Hematol, Inst Invest Biomed August Pi & Sunyer, Barcelona, Spain
关键词
PROGNOSTIC SCORING SYSTEM; INTERNATIONAL WORKING GROUP; STEM-CELL TRANSPLANTATION; POST-ESSENTIAL THROMBOCYTHEMIA; AGNOGENIC MYELOID METAPLASIA; IDIOPATHIC MYELOFIBROSIS; SPLENIC IRRADIATION; MYELOPROLIFERATIVE NEOPLASMS; LENALIDOMIDE THERAPY; POLYCYTHEMIA-VERA;
D O I
10.1182/blood-2014-07-575373
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Myelofibrosis (MF) is a BCR-ABL1-negative myeloproliferative neoplasm characterized by clonal myeloproliferation, dysregulated kinase signaling, and release of abnormal cytokines. In recent years, important progress has been made in the knowledge of the molecular biology and the prognostic assessment of MF. Conventional treatment has limited impact on the patients' survival; it includes a wait-and-see approach for asymptomatic patients, erythropoiesis-stimulating agents, androgens, or immunomodulatory agents for anemia, cytoreductive drugs such as hydroxyurea for the splenomegaly and constitutional symptoms, and splenectomy or radiotherapy in selected patients. The discovery of the Janus kinase (JAK) 2 mutation triggered the development of molecular targeted therapy of MF. The JAK inhibitors are effective in both JAK2-positive and JAK2-negative MF; one of them, ruxolitinib, is the current best available therapy for MF splenomegaly and constitutional symptoms. However, although ruxolitinib has changed the therapeutic scenario of MF, there is no clear indication of a disease-modifying effect. Allogeneic stem cell transplantation remains the only curative therapy of MF, but due to its associated morbidity and mortality, it is usually restricted to eligible high-and intermediate-2-risk MF patients. To improve current therapeutic results, the combination of JAK inhibitors with other agents is currently being tested, and newer drugs are being investigated.
引用
收藏
页码:2635 / 2642
页数:8
相关论文
共 50 条
  • [31] How would I treat?
    Vranckx, Pascal
    Valgimigli, Marco
    Heidbuchel, Hein
    EUROINTERVENTION, 2016, 12 (07) : 932 - 932
  • [32] How would I treat?
    Ferrara, Angela
    De Bruyne, Bernard
    EUROINTERVENTION, 2015, 11 (01) : 127 - 127
  • [33] How would I treat?
    van Sambeek, Marc R. H. M.
    Tielbeek, Alexander V.
    Teijink, Joep A. W.
    Cuypers, Philippe W.
    EUROINTERVENTION, 2014, 9 (09) : 123 - 123
  • [34] How would I treat?
    Kovac, Jan
    Baron, Julia
    Chin, Derek T.
    EUROINTERVENTION, 2014, 10 (01) : 172 - 172
  • [35] HOW I TREAT ERYTHRASMA
    TEREZAKIS, N
    POSTGRADUATE MEDICINE, 1970, 48 (02) : 201 - +
  • [36] How I treat dysfibrinogenemia
    Casini, Alessandro
    de Moerloose, Philippe
    BLOOD, 2021, 138 (21) : 2021 - 2030
  • [37] How to Treat Algodystrophy and Rheumatic Comorbidity in Myelofibrosis: Three Case Reports
    Magazzino, Olga
    Urbano, Tiziana
    Magnasco, Salvatore
    CUREUS JOURNAL OF MEDICAL SCIENCE, 2022, 14 (08)
  • [38] How I treat pinworms in rodents
    Messonnier, S
    EXOTIC PET PRACTICE, 1998, 3 (10): : 76 - 76
  • [39] How I treat multiple myeloma
    Busby, Leslie T.
    JOURNAL OF COMMUNITY AND SUPPORTIVE ONCOLOGY, 2016, 14 (05): : 235 - 238
  • [40] HOW I TREAT HERPES SIMPLEX
    GOLDSCHMIDT, H
    POSTGRADUATE MEDICINE, 1968, 44 (06) : 177 - +