Grunenberg Alexander, Buske Christian
Dtsch Med Wochenschr. 2016 Feb;141(3):170-2. doi: 10.1055/s-0041-107960. Epub 2016 Feb 3.
Waldenström's Macroglobulinemia (WM) is a rare subtype of B-cell lymphoma with mostly indolent course. Until now the backbone of therapy is still Rituximab / Chemotherapy. In recent years whole genome sequencing has revealed a number of genetic mutations, among them mutations of the MYD88 gene which occurs in over 90 % of patients and helps to validate diagnosis of WM in difficult cases. With the introduction of ibrutinib, an oral bruton-tyrosine-kinase inhibitor, an effective chemotherapy-free treatment option is available.
华氏巨球蛋白血症(WM)是一种罕见的B细胞淋巴瘤亚型,病程大多进展缓慢。到目前为止,治疗的主要方法仍然是利妥昔单抗/化疗。近年来,全基因组测序揭示了许多基因突变,其中MYD88基因的突变在超过90%的患者中出现,有助于在疑难病例中确诊WM。随着口服布鲁顿酪氨酸激酶抑制剂伊布替尼的引入,出现了一种有效的无化疗治疗选择。