AL amyloidosis with a localized B cell neoplasia
AL amyloidosis with a localized B cell neoplasia
复制标题
AL 淀粉样变性伴局部 B 细胞肿瘤
作者：
Christiane Stuhlmann;S. Schönland;U. Hegenbart;I. Oschlies;Julius;S. Krüger;C. Röcken
免疫球蛋白轻链衍生（AL）淀粉样变性可能作为一种全身性疾病发生，通常预后不良，而局部变异则预后良好。我们报告了 29 名患有 AL 淀粉样变性和与淀粉样沉积物空间相关的淋巴浆细胞浸润的患者。在 17 例中，淀粉样蛋白沉积物被分类为 ALλ，12 例被分类为 ALκ。所有病例的组织病理学均显示相对稀疏的浆细胞和 B 细胞，没有淋巴浆细胞浸润形成肿瘤或片层。 B 细胞主要表现出边缘区的免疫表型。原位杂交显示 17 例具有 λ-，10 例具有 κ 轻链限制性浆细胞，这与每种情况下的 AL 亚型一致。在 23/29 可解释的病例中发现克隆性免疫球蛋白重可变基因 (IGHV) 或 κ 轻链重排。单个病例存在 MYD88L265P 突变。总之，我们检测到 27 例 (93%) AL 淀粉样变性病例，伴有相关轻链限制且主要是分子克隆浆细胞群。可获得 18 名患者的临床数据。 5 名患者患有系统性淋巴瘤，2 名患者患有系统性 AL 淀粉样变性。其余病例根据 AL 淀粉样变性和轻链限制性浆细胞群分类为局部病例。据我们所知，我们在此提出了与轻链限制且主要是分子克隆浆细胞群相关的最大的 AL 淀粉样变性队列，我们将其指定为一个独特的疾病实体：“具有未确定意义的局部 B 细胞肿瘤的 AL 淀粉样变性”。
Immunoglobulin light chain-derived (AL) amyloidosis may occur as a systemic disease usually with dismal prognosis and a localized variant with favorable outcome. We report 29 patients with AL amyloidosis and associated lymphoplasmacytic infiltrate spatially related to amyloid deposits. In 17 cases, the amyloid deposits were classified as ALλ and 12 as ALκ Histopathology in all cases showed relatively sparse plasma cells and B cells without tumor or sheet formation by the lymphoplasmacytic infiltrate. The B cells predominantly showed an immunophenotype of the marginal zone. In situ, hybridization revealed 17 cases with λ− and 10 with κ light chain restricted plasma cells, which was concordant with the AL subtype in each case. Clonal immunoglobulin heavy variable gene (IGHV) or κ light chain rearrangement was found in 23/29 interpretable cases. A single case harbored a MYD88L265P-mutation. Taken together, we detected 27 (93%) cases of AL amyloidosis with an associated light chain restricted and predominantly molecularly clonal plasma cell population. Clinical data were available in 18 patients. Five patients suffered from systemic lymphoma and two from systemic AL amyloidosis. The remaining cases were classified as localized with regard to both, the AL amyloidosis and the light chain restricted plasma cell population. To the best of our knowledge, we herein present the largest cohort of AL amyloidosis associated with a light chain restricted and predominantly molecularly clonal plasma cell population, which we designate as a distinct disease entity: “AL amyloidosis with a localized B cell neoplasia of undetermined significance”.
影响因子：
20.3
作者：
Xu, Lian;Hunter, Zachary R.;Treon, Steven P.
通讯作者：
Treon, Steven P.