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新诊断多发性骨髓瘤和冒烟型骨髓瘤患者在诊断性骨髓活检时无症状免疫球蛋白轻链淀粉样变性 (AL)。一组 144 例病例和文献复习。

Asymptomatic immunoglobulin light chain amyloidosis (AL) at the time of diagnostic bone marrow biopsy in newly diagnosed patients with multiple myeloma and smoldering myeloma. A series of 144 cases and a review of the literature.

机构信息

Cattedra ed U.O. di Ematologia conTrapianto, Azienda Ospedaliera Universitaria Policlinico di Palermo, via del Vespro 127, 90127, Palermo, Italy.

出版信息

Ann Hematol. 2011 Jan;90(1):101-6. doi: 10.1007/s00277-010-1028-8. Epub 2010 Jul 20.

Abstract

The rate of asymptomatic amyloidosis (AL) among patients with newly diagnosed multiple myeloma (MM) or smoldering multiple myeloma (SMM) is unknown. We evaluated number and clinical significance of asymptomatic AL in consecutive MM and SMM patients, not having recognition of symptomatic AL at the time of their diagnostic bone marrow biopsy. Bone marrow biopsies were stained with Congo red and considered diagnostic for AL in case of positive Congo red staining with apple-green birefringence. Biopsies from 144 patients were evaluated: 77 had a diagnosis of MM and 67 of SMM. The median age was 59 (range 26-84) years; the median follow-up was 76 months (range 0-216). Immunoglobulin isotypes were 96/144 (67%), IgG; 23/144 (16%), IgA; 12/144 (8%), light chain only; 1/77 (1%), IgD; and biclonal or indeterminate, 12/144 (8%). Fifty-eight percent (84/144) were κ restricted. The presence of amyloid was found in two cases (1%, 95% CI -0.6 to 3.2), one in MM, and one in SMM group, and none had or developed signs or symptoms suggestive of organ involvement by amyloid. Among the 142 other patients without amyloid deposition in their index bone marrow, one (0.7%, 95% CI -0.6 to 2.0) developed symptomatic AL after 119 months.

摘要

新诊断的多发性骨髓瘤(MM)或冒烟型多发性骨髓瘤(SMM)患者无症状淀粉样变性(AL)的发生率尚不清楚。我们评估了连续 MM 和 SMM 患者中无症状 AL 的数量和临床意义,这些患者在进行诊断性骨髓活检时没有出现有症状 AL 的迹象。骨髓活检用刚果红染色,如果刚果红染色呈阳性,出现苹果绿双折射,则认为具有 AL 诊断意义。评估了 144 例患者的活检标本:77 例诊断为 MM,67 例诊断为 SMM。中位年龄为 59 岁(范围 26-84 岁);中位随访时间为 76 个月(范围 0-216 个月)。免疫球蛋白同种型为 96/144(67%),IgG;23/144(16%),IgA;12/144(8%),仅轻链;1/77(1%),IgD;双克隆或不确定,12/144(8%)。58%(84/144)为κ限制性。在 2 例(1%,95%CI-0.6 至 3.2)患者中发现淀粉样物质,1 例为 MM,1 例为 SMM 组,均无或无器官受累的迹象或症状提示淀粉样变性。在其余 142 例骨髓中未发现淀粉样物质沉积的患者中,1 例(0.7%,95%CI-0.6 至 2.0)在 119 个月后发生了有症状的 AL。

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