Matsuda Masayuki, Gono Takahisa, Shimojima Yasuhiro, Yoshida Takuhiro, Katoh Nagaaki, Hoshii Yoshinobu, Yamada Toshiyuki, Ikeda Shu-ichi
Department of Medicine (Neurology and Rheumatology, Shinshu University School of Medicine, Matsumoto, Japan.
Amyloid. 2008 Jun;15(2):117-24. doi: 10.1080/13506120802006047.
We report three patients with AL amyloidosis manifesting as systemic lymphadenopathy, mainly in the cervical and supraclavicular regions. Histopathology of lymph nodes showed massive deposition of AL amyloid with no abnormal findings suggestive of lymphoproliferative disorders. Two of the patients were considered to be classifiable as primary systemic AL amyloidosis based on the presence of M-protein in serum and abnormal plasma cells or lymphoplasmacytoid cells in the bone marrow probably producing the precursor immunoglobulin, although no visceral organs were affected. The size of the involved lymph nodes in these two patients increased gradually, and one was treated with rituximab and VAD (vincristine, doxorubicin and dexamethasone) followed by high-dose melphalan with autologous peripheral blood stem cell transplantation (auto-PBSCT). The remaining patient showed no obvious change in the size of lymph nodes or detectable M-protein in serum. The prognosis of AL amyloidosis manifesting as lymphadenopathy is usually good as long as there are no hematological malignancies or rapid increases in the size of lymph nodes, but in cases of the systemic type, intensive chemotherapy, such as high-dose melphalan with auto-PBSCT, should be actively considered in order to avoid possible involvement of visceral organs.
我们报告了3例以全身淋巴结肿大为主要表现的AL淀粉样变性患者，肿大淋巴结主要位于颈部和锁骨上区域。淋巴结组织病理学检查显示有大量AL淀粉样蛋白沉积，未发现提示淋巴增殖性疾病的异常表现。其中2例患者血清中存在M蛋白，骨髓中存在可能产生前体免疫球蛋白的异常浆细胞或淋巴浆细胞样细胞，尽管没有内脏器官受累，但仍被认为可归类为原发性系统性AL淀粉样变性。这2例患者受累淋巴结大小逐渐增大，其中1例接受了利妥昔单抗和VAD（长春新碱、阿霉素和地塞米松）治疗，随后进行了大剂量美法仑联合自体外周血干细胞移植（auto-PBSCT）。其余1例患者淋巴结大小无明显变化，血清中未检测到M蛋白。只要不存在血液系统恶性肿瘤或淋巴结大小迅速增加，以淋巴结肿大为表现的AL淀粉样变性的预后通常较好，但对于系统性类型的病例，应积极考虑进行强化化疗，如大剂量美法仑联合auto-PBSCT，以避免内脏器官可能受累。
