Landeiro Luís, Freitas Ana Carolina, Proença Margarida, Cabeçadas José, Nunes Albertina, Bayão Horta Alexandra
Internal Medicine Department, Hospital da Luz, Lisboa, Portugal.
Hematology Department, Instituto Português de Oncologia Francisco Gentil, Lisboa, Portugal.
Eur J Case Rep Intern Med. 2021 Oct 20;8(10):002831. doi: 10.12890/2021_002831. eCollection 2021.
Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients the aetiology is still unknown, hence the designation of idiopathic MCD (iMCD). To successfully diagnose iMCD, diagnostic criteria must be fulfilled and a large array of alternative diagnoses excluded. Peripheral neuropathy and nephropathy are relatively common findings in cases associated with POEMS syndrome, but very rarely reported in iMCD. We present the case of a 64-year-old man with iMCD (HIV- and HHV-8-negative) with nephrotic syndrome and severe motor polyneuropathy. Alternative diagnoses were excluded. The patient was treated with intravenous glucocorticoid followed by rituximab. Complete clinical and laboratory remission was achieved and maintained at the 2-year follow-up.
iMCD is a lymphoproliferative disease in immunocompetent patients with no known cause.To diagnose iMCD major and minor criteria must be fulfilled, and alternative diagnoses must be excluded.Nephrotic syndrome and motor polyneuropathy are rare in iMCD and all alternative diagnoses must be excluded before relating all of these.
多中心Castleman病（MCD）是一组与促炎性高细胞因子血症相关、了解甚少的淋巴增殖性疾病。在免疫功能正常的患者中，病因仍不明，因此称为特发性MCD（iMCD）。要成功诊断iMCD，必须满足诊断标准并排除一系列其他诊断。周围神经病变和肾病在与POEMS综合征相关的病例中相对常见，但在iMCD中报道极少。我们报告一例64岁患有iMCD（HIV和HHV - 8阴性）的男性患者，伴有肾病综合征和严重运动性多发性神经病变。已排除其他诊断。患者先接受静脉糖皮质激素治疗，随后使用利妥昔单抗。实现了完全的临床和实验室缓解，并在2年随访中维持。
iMCD是免疫功能正常患者中病因不明的淋巴增殖性疾病。诊断iMCD必须满足主要和次要标准，并排除其他诊断。肾病综合征和运动性多发性神经病变在iMCD中罕见，在将所有这些情况联系起来之前必须排除所有其他诊断。
