Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA 02115, USA.
Clin Lymphoma Myeloma Leuk. 2011 Jun;11 Suppl 1(Suppl 1):S65-9. doi: 10.1016/j.clml.2011.03.022. Epub 2011 Apr 30.
Waldenstrom macroglobulinemia (WM) is a low-grade B-cell lymphoma characterized by widespread involvement of the bone marrow with lymphoplasmacytic cells. In approximately 20% of patients, the malignant clone also involves the lymph nodes and induces hepatosplenomegaly. The mechanisms by which the tumor cells home to the bone marrow and preferentially reside in the marrow niches are not fully elucidated. In this review, we examine the role of the bone marrow microenvironment in the regulation of cell growth, survival and cell dissemination in WM. We also summarize specific regulators of niche-dependent tumor proliferation in WM. These include chemokines, adhesion molecules, Src/PI3K/Akt/mTOR signaling, NF-kB activation, and micro-RNA regulation in WM. Targeting these pathways in clinical trials could lead to significant responses in this rare disease.
华氏巨球蛋白血症（WM）是一种低级别 B 细胞淋巴瘤，其特征是骨髓中广泛浸润淋巴浆细胞。大约 20%的患者，恶性克隆也会累及淋巴结并引起肝脾肿大。肿瘤细胞归巢到骨髓并优先驻留在骨髓龛中的机制尚未完全阐明。在这篇综述中，我们研究了骨髓微环境在 WM 中调节细胞生长、存活和细胞播散的作用。我们还总结了 WM 中调节龛依赖性肿瘤增殖的特定调节剂。这些调节剂包括趋化因子、黏附分子、Src/PI3K/Akt/mTOR 信号通路、NF-κB 激活和 micro-RNA 调节。在临床试验中靶向这些通路可能会导致这种罕见疾病的显著反应。
