waldenström巨球蛋白血症的不典型临床表现:诊断困难

E. F. Makhnyr’, N. Shostak, N. Inasaridze, E. V. Chernaya
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引用次数: 2

摘要

目标。描述诊断的困难Waldenström巨球蛋白血症患者的非典型临床表现。材料和方法。患者K, 57岁,因右侧单侧下颌下淋巴结增多,鼻咽不适,咳嗽无痰,疲劳加重而就诊。在检查期间,向她咨询的有:传染病专家、耳鼻喉科医生、外科医生、牙医、眼科医生、血液科医生和肿瘤科医生。为确认诊断进行了诊断活动:实验室参数动态评估,检查方案癌症搜索(包括多层计算机断层扫描和各区域磁共振成像),除结核病外,单基因研究血液和尿液中的蛋白质检查,回肠活检,免疫组织化学trepanobbiopsy。结果:在患者4年的血液检查中,检测到γ区m梯度,单克隆免疫球蛋白М-κ 19.3 g / l,免疫固定期间检测尿中ben - jones -κ蛋白(每日蛋白尿0.45 g)。在光光学水平的骨髓图中,细胞成分的数量明显减少,淋巴细胞增生明显40%,粒细胞系列和红细胞相对狭窄,浆细胞6%。我们根据病人的免疫形态学图片,根据实验室的数据研究方法,对应于Waldenström病中骨髓中-κ的失败,分泌m -副蛋白。本-琼斯蛋白在尿液中的检测是本-琼斯蛋白在发病时是不存在的,但在肿瘤淋巴细胞增生方面却几乎是典型的。结论本病例的有趣之处不仅在于Waldenström巨球蛋白血症的诊断的复杂性,而且在于本例患者的不典型、隐匿性临床疾病。
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Atypical clinical picture of waldenström’s macroglobulinemia: a difficult path to diagnosis
Objective.To describe the difficulties of diagnosis of Waldenström macroglobulinemia in a patient with atypical clinical picture.Materials and methods.Patient K., 57 years old, came in outpatient department with complaints of unilateral increase in submandibular lymph nodes on the right, discomfort in the nasopharynx, cough without sputum, increased fatigue. During the examination she was consulted by: an infectious disease specialist, otolaryngologist, surgeon, dentist, phthisiologist, hematologist and oncologist consulted her. To confirm the diagnosis conducted diagnostic activities: dynamic assessment of laboratory parameters, examination program cancer search (including multislice computed tomography and magnetic resonance imaging of various areas), with the exception of tuberculosis, monogenically study proteins in the blood and urine tests, biopsy of the ileum, immunohistochemistry trepanobiopsy.Results.During the 4‑year examination in the patient’s blood, an M-gradient in the gamma zone was detected, monoclonal immunoglobulin М-κ 19.3 g / l, Bens-Jones-κ protein in urine (daily proteinuria 0.45 g) was detected during immunofixation. In the myelogram at the light-optical level, the number of cell elements was significantly reduced with a pronounced lymphoid proliferation of 40 %, the granulocytic series and erythropoiesis were relatively narrowed, and plasma cells 6 %. Immunomorphological picture trepanobiopsy our patient based on the data of laboratory methods of research corresponds to the defeat of the bone marrow in Waldenström’s disease-κ, secretion of M-paraprotein. An important feature that allowed to go on the right path of diagnosis was almost pathognomonic for tumor lymphoproliferation, detection of Bens-Jones protein in the urine, which was absent in the onset of the disease.Conclusion.This clinical case is interesting not only by the complexity of the diagnosis of macroglobulinemia of Waldenström in General, but also by the atypical, erased clinical disease in our patient.
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