Clinical Analysis of Unicentric Castleman's Disease with Paraneoplastic Pemphigus and Bronchiolitis Obliterans.
| Autor: | Zhen JF; Department of International Medical Services, ,CAMS and PUMC,Beijing 100730,China., Zhang L; Department of Hematology,PUMC Hospital, CAMS and PUMC,Beijing 100730,China., Cao XX; Department of Hematology,PUMC Hospital, CAMS and PUMC,Beijing 100730,China., Feng J; Department of Hematology,PUMC Hospital, CAMS and PUMC,Beijing 100730,China., Zhou DB; Department of Hematology,PUMC Hospital, CAMS and PUMC,Beijing 100730,China., Lin SB; Department of International Medical Services, ,CAMS and PUMC,Beijing 100730,China., Li J; Department of Hematology,PUMC Hospital, CAMS and PUMC,Beijing 100730,China. |
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| Jazyk: | angličtina |
| Zdroj: | Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae [Zhongguo Yi Xue Ke Xue Yuan Xue Bao] 2017 Aug 20; Vol. 39 (4), pp. 492-498. |
| DOI: | 10.3881/j.issn.1000-503X.2017.04.006 |
| Abstrakt: | Objective To investigate the clinical features of unicentric Castleman's disease(UCD)with paraneoplastic pemphigus(PNP)and bronchiolitis obliterans(BO).Method Data of UCD patients with PNP and BO from Peking Union Medical College Hospital were retrospectively analyzed,along with literatures review. Results Totally 23 cases(11 males and 12 females)were enrolled.The median age was 31 years(13-56 years).The most common pathological type was hyaline-vascular variant(91.4%),and most tumors located in abdominopelvic cavity(69.6%).Considerable cases presented bulky masses(26.3%).Most cases were first diagnosed on presentation with the symtoms of PNP(90.0%).BO was characterized by progressive dyspnea after excision of CD lesions.The average follow-up duration was 27.5 months(1-135 months).The median overall survival time was 36.0 months(95% CI=13.9-58.1).Respiratory failure was the dominant cause of death(91.7%).Conclusions PNP should be considered among those patients with specific oral or cutaneous lesions.Earlier diagnosis and treatment of latent UCD are important for reducing complications and deaths. |
| Databáze: | MEDLINE |
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