[局限性AL淀粉样变]。

IF 0.3 4区 医学 Q3 MEDICINE, GENERAL & INTERNAL
N A Kashchavtseva, I G Rekhtina, A M Kovrigina, T P Danilina, L P Mendeleeva
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引用次数: 0

摘要

摘要局部轻链淀粉样变性(淀粉样瘤)是一种罕见的疾病,其发病机制尚不清楚,治疗方法尚不成熟。本文报告3例局限性AL淀粉样变伴呼吸道、眼睛和面部软组织病变的临床观察。肿瘤团块由淀粉样蛋白团块组成,其中“嵌入”了单个的单型浆细胞。为了验证细胞底物,采用免疫球蛋白轻链探针显色原位杂交的方法。在石蜡切片经pronase酶处理后,使用fitc标记的kappa和lambda轻链抗体进行淀粉样蛋白分型。缺乏单克隆分泌(在两个病例中)使得难以评估治疗的疗效。考虑到重要器官损害的普遍性和严重程度,以及疾病过程的复发性,规定了与靶向药物的全身联合治疗。在一年的随访中,所有患者的过程稳定。然而,克隆减少治疗局限性AL淀粉样变性不被认为是一种标准的选择,其可行性和有效性有待进一步证实。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Localized AL amyloidosis].

Localized light chain (AL) amyloidosis (amyloidoma) is a rare disease with unclear pathogenesis and undeveloped approaches to therapy. This article presents 3 clinical observations of localized AL amyloidosis with lesions of the respiratory tract, eyes, and soft tissues of the face. Tumor masses consisted of amyloid masses in which single monotypic plasma cells were "embedded". To verify the cell substrate, the method of chromogenic in situ hybridization with probes to immunoglobulin light chains was used. Amyloid typing was performed using FITC-labeled antibodies to kappa and lambda light chains after enzymatic treatment of paraffin sections with pronase. The absence of monoclonal secretion (in two cases) made it difficult to assess the efficacy of the therapy. Taking into account the prevalence and severity of vital organs damage, as well as the recurrent nature of the disease course, systemic combination therapy with targeted drugs was prescribed. Stabilization of the process in all patients was stated during one year of follow-up. Nevertheless, clonreducing therapy in localized AL amyloidosis is not considered as a standard option, its feasibility and efficacy requires further confirmation.

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来源期刊
Terapevticheskii Arkhiv
Terapevticheskii Arkhiv 医学-医学:内科
CiteScore
1.40
自引率
33.30%
发文量
171
审稿时长
3-8 weeks
期刊介绍: Терапевтический архив The journal was founded by the prominent Russian therapists M.P. Konchalovsky and G.F. Lang in 1923. Then its editors-in-chief were Professors V.N. Vinogradov and A.G. Gukasyan. Since 1972, E.I. Chazov, Academician of the Russian Academy of Sciences, has been heading the editorial board of the journal. Over 90 years, there have been more than 1000 issues where the authors and editorial staff have done their best for readers to keep abreast of current advances in medical science and practice and for physicians to master the advanced principles of recognition and treatment of a wide spectrum of visceral diseases. The papers published in the journal (editorials, original articles, lectures, reviews, etc.) cover both current scientific achievements and practical experience in diagnosing, treating, and preventing visceral diseases. The authors of publications are not only Russian, but also foreign scientists and physicians. All papers are peer-reviewed by highly qualified Russian specialists. The journal is published monthly. Traditionally, each issue has predominantly certain thematic areas covering individual therapy specializations. Every year, one of the issues is devoted to related problems in practical medicine (allergology and immunology, neurology and psychiatry, obstetrics, oncology, etc.). This all draws the attention of the reading public to the journal. The journal is indexed in RSCI (Russian Science Citation Index), PubMed/Medline, Index Medicus, Scopus/EMBASE, Web of Science Core Collection (Science Citation Index Expanded), Web of Science (Russian Science Citation Index - RSCI, Current Contents Connect, BIOSIS Previews), Google Scholar, Ulrich''s Periodicals Directory. The journal is included in the list of periodicals recommended by the Higher Attestation Committee for publishing the papers containing the basic materials of doctoral and candidate dissertations. By the decision of the Presidium of the Russian Academy of Medical Sciences, the “Therapevticheskiy Arkhiv” was awarded the Botkin medal. It was admitted to the European Association of Sciences Editors (EASE). The journal was honored with the Golden Press Fund decoration at the 13th International Press Professional Exhibition.
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