Lymphoproliferative disorder – Castleman’s disease with early childhood-onset in a child (a clinical case)

IF 0.1 Q4 MEDICINE, GENERAL & INTERNAL
O. Mukvich, N. Vdovina, H. Klymniuk, L. Omelchenko, T. A. Hridina, N. B. Matsiuk
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引用次数: 0

Abstract

Castleman’s disease (angiofollicular lymphoma, giant lymph node hyperplasia, Castleman’s pseudotumor) is an understudied orphan lymphoproliferative disease with a long period of asymptomatic course and a high risk of malignancy, and variability of its clinical features can cause difficulties in diagnosis. The aim of the study. To acquaint clinicians and increase the effectiveness of early diagnosis and treatment of Castleman’s disease (CD) in children to prevent malignancy and improve prognosis. Results. The article presents a clinical case of CD in a 3.5-year-old boy with a long history of fever, stunted growth, sweating, arthralgia, anemia, high laboratory inflammatory factors, elevated IL-6. There was a complex diagnostic search except for infectious, immunodeficiency, autoimmune conditions. The diagnosis of hyaline-vascular type of CD was confirmed by immunohistochemical examination after lymph node excisional biopsy. Rapid positive dynamics was noted after radical removal of the lymph node conglomerate. At follow-up after 8 months, the patient met criteria for clinical and laboratory remission. Conclusions. Castleman’s disease should be included in the search algorithm for lymphoproliferative diseases. Determining the role of genetic mutations in interferon regulatory factor (IRF8) is of scientific interest to clarify the etiology of this disease.
淋巴增生性疾病-儿童早期发病的Castleman病(1例临床)
Castleman病(血管滤泡性淋巴瘤、巨大淋巴结增生、Castleman假瘤)是一种孤儿性淋巴增生性疾病,病程长,无症状,恶性风险高,临床特征的多变性给诊断带来困难。研究的目的。目的:了解临床医生,提高儿童Castleman病(CD)早期诊断和治疗的有效性,预防恶性肿瘤,改善预后。本文报告一例3.5岁男孩乳糜泻临床病例,有长期发热、发育迟缓、多汗、关节痛、贫血、实验室炎性因子高、IL-6升高等病史。有一个复杂的诊断搜索,除了传染性,免疫缺陷,自身免疫性疾病。淋巴结切除活检后免疫组化检查证实透明血管型CD的诊断。淋巴结砾岩根治性切除后出现快速阳性动态。8个月后随访,患者符合临床和实验室缓解标准。应将Castleman病纳入淋巴增生性疾病的搜索算法中。确定干扰素调节因子(IRF8)基因突变的作用对阐明该病的病因具有重要的科学意义。
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来源期刊
Zaporozhye Medical Journal
Zaporozhye Medical Journal MEDICINE, GENERAL & INTERNAL-
自引率
0.00%
发文量
72
审稿时长
8 weeks
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