Q4 Medicine Hematologia Pub Date : 2019-03-04 DOI:10.5603/HEM.2018.0040
Michał Bator, S. Potoczek, L. Usnarska-Zubkiewicz
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引用次数: 0

摘要

我们报告了一位69岁的患者,他于2016年3月被诊断为意义不明的单克隆γ病(MGUS)。该病是在诊断血小板减少症时发现的(从2009年起,血小板数量在90-140 G/l之间变化)。血清免疫固定试验证实存在单克隆IgG kappa蛋白。2017年7月,当患者出现肾病综合征和急性肾功能衰竭时,骨髓中发现14.5%的浆细胞。此外,腹部脂肪活检显示存在由kappa轻链组成的淀粉样蛋白。建立IgG κ浆细胞骨髓瘤(PCM)合并肾、心、肝、脾淀粉样变的诊断。PCM合并淀粉样变的进展非常具有侵略性。尽管给予硼替佐米和地塞米松治疗,患者仍早死。MGUS患者的突然健康恶化(如急性肾衰竭)不仅表明MGUS向PCM发展,而且还表明淀粉样变的发展,这与疾病动荡和预后不良有关。
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Od monoklonalnej gammapatii o nieokreślonym znaczeniu (MGUS) do szpiczaka plazmocytowego i amyloidozy — opis przypadku
We present a 69-year-old patient, in whom in March 2016 a monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. The disease was found during the diagnostics of thrombocytopenia (from 2009 the number of platelets varied between 90–140 G/l). In serum immunofixation assay the presence of monoclonal IgG kappa protein was confirmed. In July 2017, when the patient presented with nephrotic syndrome and acute renal failure, 14.5% of plasma cells were found in the bone marrow. Moreover, a biopsy of abdominal fat revealed the presence of amyloid composed of kappa light chains. The diagnosis of IgG kappa plasma cell myeloma (PCM) complicated with amyloidosis of the kidneys, heart, liver and spleen was established. The PCM progression complicated with amyloidosis was very aggressive. Despite the treatment with bortezomib and dexamethasone the patient died early. Sudden deterioration of health (e.g. in the form of acute renal failure) in a patient with MGUS may indicate not only the progression of MGUS to PCM, but also the development of amyloidosis, which is associated with turbulent disease and poor prognosis.
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来源期刊
Hematologia
Hematologia Medicine-Oncology
自引率
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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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