Multicentric Castleman's disease combined with polyserositis and POEMS syndrome: case report and review article

被引：1

作者：

Ulas, Sevtap Tugce ^{[1
]}

Dasdelen, Suha ^{[2
,3
]}

机构：

[1] Charite Univ Med Berlin, Klin Radiol, Campus Mitte, Berlin, Germany

[2] Vivantes Humboldt Klinikum, Klin Nephrol, Nordgraben 2, D-13509 Berlin, Germany

[3] Private Univ Klin Witten Herdecke, Witten, Germany

来源：

INTERNIST | 2021年 / 62卷 / 07期

关键词：

Multicentric Castleman's disease; Monoclonal gammopathy of undetermined significance; Vascular endothelial growth factor (VEGF); Interleukin-6; Antibodies; monoclonal; HYALINE VASCULAR-TYPE; GROWTH-FACTOR LEVELS; MONOCLONAL-ANTIBODY; MANIFESTATIONS; PATHOGENESIS; SILTUXIMAB; SPECTRUM; THERAPY; BIOLOGY;

D O I：

10.1007/s00108-021-01063-4

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Castleman disease (CD) is a very rare disorder characterised by hyperplasia of the lymphoid tissue. The aetiology varies considerably and includes autoimmunological, infectious, autoinflammatory and paraneoplastic diseases (e.g. MGUS with POEMS syndrome). What they all have in common is usually a dysregulation/overproduction of certain cytokines and growth factors (including interleukin 6 and VEGF). The sum of these changes sometimes causes very heterogeneous symptoms and thus often makes early diagnosis difficult. The prognosis of unrecognised and untreated disease is very serious and has an average 5-year survival rate of 55-77%. The present paper describes the case of a 79-year-old patient with refractory polyserositis who was correctly diagnosed after > 8 years.

引用

页码：777 / 785

页数：9

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