JYC Chan, QU Lee, SC Ling, CK Li, NKC Tse

Abstract

Castleman disease (CMD) is an uncommon lymphoproliferative disorder characterised by hyperplasia of lymphoid tissue. There are three histopathologic types of CMD: hyaline-vascular type, plasma cell type and mixed type. Hyaline-vascular CMD is typically unicentric, while plasma cell CMD tends to be multicentric. In rare occasion, CMD can be associated with paraneoplastic pemphigus (PNP), an autoimmune syndrome which encompasses a multitude of mucocutaneous and systemic clinical features. In children with PNP associated with Castleman disease, they have been reported to have very poor prognosis and high mortality rate as a result of respiratory compromise despite aggressive treatment. We report a case of unicentric, CD-20 positive, hyaline-vascular retroperitoneal CMD presenting with paraneoplastic pemphigus and bronchiolitis obliterans with significant clinical improvement in oral mucositis and the prevention of tumour recurrence, using regular rituximab (375 mg/m²/dose) and 3-day course of pulse methylprednisolone therapy (10 mg/kg/day) after tumour resection.

Castleman病（CMD）是一種少見的淋巴組織增生異常性疾病，以淋巴組織過度增殖為特點。根據病理類型，CMD分為透明血管型、漿細胞型以及混合型。透明血管型CMD為典型單中心型，而漿細胞型則為多中心型。極少情況下，CMD會伴發出現副腫瘤性天皰瘡（PNP），一種出現皮膚粘膜以及全身系統臨床症狀的自身免疫異常綜合徵。過往報導兒童CMD伴PNP的預後非常差，即使採取積極治療仍會因出現呼吸窘迫而有很高的病死率。我們報導了一例單中心型，CD20 陽性，透明血管型的腹膜後CMD同時伴有PNP以及閉塞性細支氣管炎的病人，經予以病人Rituximab（375 mg/m2/dose）以及於手術切除腫瘤之後，給予3天療程的甲基潑尼松龍衝擊治療（10 mg/kg/day）之後，患兒口腔粘膜炎顯著改善的同時預防了腫瘤復發。