Abstract
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.
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Chen, Z., Chen, Z., Wu, S. et al. Niemann-Pick disease resulting in spontaneous splenic rupture in an adult: Report of a case. Surg Today 38, 473–475 (2008). https://doi.org/10.1007/s00595-007-3641-2
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DOI: https://doi.org/10.1007/s00595-007-3641-2