[华氏巨球蛋白血症]
[Waldenström macroglobulinaemia].
作者信息
Tomowiak Cécile, Poulain Stéphanie, Debiais Céline, Guidez Stéphanie, Leleu Xavier
机构信息
CHU de Poitiers, service d'oncohématologie et thérapie cellulaire, 86021 Poitiers.
CHU de Lille, laboratoire d'hématologie cellulaire, 59000 Lille.
出版信息
Presse Med. 2019 Jul-Aug;48(7-8 Pt 1):832-841. doi: 10.1016/j.lpm.2019.07.020. Epub 2019 Aug 20.
Lymphoplasmocytic lymphona with monoclonal lgM, rare. Median age at diagnosis 70 years old, frail population. Heterogenous clinic presentation. Molecular diagnosis with MYD88. Treatment required for symptomatic WM patients only. 1st line therapy: DRC. Input of targeted therapies (ibrutinib) for frail patients, maintenance effect.
伴有单克隆IgM的淋巴浆细胞淋巴瘤，罕见。诊断时的中位年龄为70岁，患者体质较弱。临床表现多样。通过MYD88进行分子诊断。仅对有症状的华氏巨球蛋白血症患者需要治疗。一线治疗：DRC。对体弱患者采用靶向治疗（伊布替尼），有维持疗效。
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