title: Successful treatment of kaposiform lymphangiomatosis with sirolimus.
abstract: Generalized lymphatic anomaly (GLA) is a rare and often fatal congenital lymphatic disorder that also commonly affects bone. Kaposiform lymphangiomatosis (KLA) is a novel subtype of GLA with poor prognosis and no proper treatment guidelines. A 9-year-old male with recurrent pleural effusion was clinically diagnosed as KLA. Following sirolimus therapy at a dose of 0.8 mg/m(2) twice daily, pleural effusion was significantly decreased and the general status of the patient markedly improved. The clinical course indicates that sirolimus may present an effective therapeutic option in KLA. Moreover, KLA should be considered in differential diagnosis for cases of GLA with coagulopathy.
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
authors.forename: Zuopeng
authors.initials: Z
authors.lastname: Wang
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
authors.forename: Kai
authors.initials: K
authors.lastname: Li
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
authors.forename: Wei
authors.initials: W
authors.lastname: Yao
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
authors.forename: Kuiran
authors.initials: K
authors.lastname: Dong
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
authors.forename: Xianmin
authors.initials: X
authors.lastname: Xiao
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
authors.forename: Shan
authors.initials: S
authors.lastname: Zheng
mesh_terms: D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use*; D002648:Child; D006390:Hemangioendothelioma / Q000188:drug therapy*; D006801:Humans; D059885:Kasabach-Merritt Syndrome / Q000188:drug therapy*; D008297:Male; D010996:Pleural Effusion / Q000517:prevention & control*; D011379:Prognosis; D012514:Sarcoma, Kaposi / Q000188:drug therapy*; D020123:Sirolimus / Q000627:therapeutic use*
chemical_list: D000903:Antibiotics, Antineoplastic; D020123:Sirolimus
keywords: generalized lymphatic anomaly; kaposiform lymphangiomatosis; sirolimus; vascular malformation