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HK J Paediatr (New Series)
Vol 16. No. 3,
2011
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HK J Paediatr (New Series) 2011;16:169-174
Case Report
Hyaline-vascular Variant Unicentric Castleman Disease with Paraneoplastic Pemphigus and Bronchiolitis Obliterans Treated with Rituximab: Case Report
JYC Chan, QU Lee, SC Ling, CK Li, NKC Tse Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, 2-10 Princess Margaret Hospital Road, Lai Chi Kok, Kowloon, Hong Kong, China JYC Chan (陳亦俊) MBChB(CUHK), MRCPCH, MPH QU Lee (李君宇) MRCP(UK), FHKAM(Paed), FHKCPaed SC Ling (凌紹祥) MRCP(UK), FHKCPaed, FHKAM(Paed) CK Li (李志強) FHKAM(Paed), FHKCPaed, FRCPCH(UK) NKC Tse (謝紀超) FHKCPaed, FHKAM(Paed), FRCP(Edin) Correspondence to: Dr JYC Chan Received February 9, 2011
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. Keyword : Bronchiolitis obliterans; Castleman disease; Children; Paraneoplastic pemphigus; Rituximab Abstract in Chinese
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