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.
Chen
Zihua
Z
Department of General Surgery, Xiangya Hospital of Central South University, Changsha, Hunan, P.R. China.
Chen
Zhikang
Z
Wu
Shaobin
S
Wang
Xianwei
X
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