Q4 Medicine Hematologia Pub Date : 2018-10-17 DOI:10.5603/HEM.2018.0025
Agnieszka Krzywdzińska, Iwona Solarska, B. Pula, K. Jamroziak
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摘要

轻链淀粉样变性(AL amyloidosis)是一种由克隆骨髓浆细胞产生的单克隆免疫球蛋白轻链淀粉样前体积累而引起淀粉样纤维沉积而导致器官损伤的全身性疾病。AL淀粉样变性治疗的目的是抑制淀粉样免疫球蛋白轻链,从而稳定甚至改善受累器官的功能。治疗效果评估是基于血液学和器官反应。尽管获得了完全的血液学缓解,但在一些患者中,即使是微量的浆细胞在治疗后持续存在,也可能导致产生少量的单克隆免疫球蛋白,从而加深器官损伤。这些浆细胞无法被常规诊断方法检测到,被认为是微小残留疾病(MRD)。考虑到目前已发表的研究结果,基于流式细胞术的MRD评估有可能成为未来AL淀粉样变性治疗疗效评估的基本工具。本文介绍了AL淀粉样变性的MRD评估方法和最新研究结果。
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Znaczenie oceny minimalnej choroby resztkowej w amyloidozie AL
Light chain amyloidosis (AL amyloidosis) is a systemic disease leading to organ damage due to deposition of amyloid fibers arising from accumulation of amyloid precursors – monoclonal immunoglobulin light chains produced by clonal bone marrow plasmocytes. The aim of AL amyloidosis therapy is the inhibition amyloidogenic immunoglobulin light chains, what should result in stabilization or often even improvement of involved organs’ function. Treatment effectiveness evaluation is based on hematological and organ response. Despite achievement of complete hematological remission, in some of the patients even a trace number of plasmocytes persisting following treatment may lead to production of low amount of monoclonal immunoglobulins capable of deepening organ damage. These plasmocytes remain undetectable to routine diagnostic approaches and are regarded as minimal residual disease (MRD). Considering the results of the so far published research, it is possible that MRD assessment based on flow cytometric technique may constitute a basic tool of response assessment of AL amyloidosis treatment in the future. In this publication the methodology and results of latest research concerning MRD assessment in AL amyloidosis are presented.
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来源期刊
Hematologia
Hematologia Medicine-Oncology
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0
审稿时长
4 weeks
期刊介绍: Hematology is the quarterly under auspices of the Institute of Hematology and Transfusion Medicine. The journal is addressed to hematologists, oncologists and also internists. It contains the overview/review articles, case reports, essays, including reports from the scientific and educational conferences as well as test questions on hematology. Journal of the Institute of Hematology and Transfusiology.
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