Niemann-Pick disease resulting in spontaneous splenic rupture in an adult: report of a case.
Niemann-Pick disease (NPD) is an inherited metabolic disorder, which has been classified into types A, B, C, and D. Niemann-Pick disease rarely causes spontaneous splenic rupture: to our knowledge, only four such cases have ever been reported. We report the case of a 53-year-old man with NPD manifesting as spontaneous splenic rupture. Ultrasound showed a ruptured large spleen with hemoperitoneum, and he underwent emergency splenectomy. Histopathological examination confirmed that there were numerous accumulated "Pick cells," defined as foamy histiocytes, in the spleen.
Department of General Surgery, Xiangya Hospital of Central South University, Changsha, Hunan, P.R. China.
Zihua
Z
Chen
Zhikang
Z
Chen
Shaobin
S
Wu
Xianwei
X
Wang
D006644:Histiocytes / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009542:Niemann-Pick Diseases / Q000150:complications*; D012422:Rupture, Spontaneous; D013154:Spleen / Q000473:pathology*; D013161:Splenic Rupture / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D014463:Ultrasonography