Priapism following splenectomy in an unstable hemoglobin: Hemoglobin Olmsted β141 (H19) Leu→Arg

Arg) This is a rare hemoglobin variant, which until now has been reported only once. The clinical course of the 12-year-old boy was characterized by severe hemolytic anemia leading to splenectomy and cholecystectomy at the of 3.5 years. The priapism occurred 8 years after splenectomy, during a hemolytic febrile episode and required aspiration of the corpora cavernosa. This report raises the question of the benefit of splenectomy in patients suffering from a chronic hemolytic anemia such as that due to an unstable hemoglobin. This treatment lowers the frequency and the severity of acute hemolytic attacks, but several cases of vascular complications have been reported after splenectomy. -->
ISSN: 1096-8652
0361-8609
DOI: 10.1002/(sici)1096-8652(199602)51:2<133::aid-ajh6>3.0.co;2-z
Přístupová URL adresa: https://explore.openaire.eu/search/publication?articleId=doi_________::f622891c0eecc14bff756ff51bf88b58
https://doi.org/10.1002/(sici)1096-8652(199602)51:2<133::aid-ajh6>3.0.co;2-z
Rights: CLOSED
Přírůstkové číslo: edsair.doi...........f622891c0eecc14bff756ff51bf88b58
Autor: Henri Perrimond, Francis Giraud, Henri Wajcman, Frédéric Galactéros, Danielle Vanuxem, Isabelle Thuret, Danielle Lena-Russo, Catherine Badens, J. Bardakdjian
Rok vydání: 1996
Předmět:
Zdroj: American Journal of Hematology. 51:133-136
ISSN: 1096-8652
0361-8609
DOI: 10.1002/(sici)1096-8652(199602)51:2<133::aid-ajh6>3.0.co;2-z
Popis: We report a case of severe priapism occurring in a patient with an unstable hemoglobin, Hb Olmsted (beta 141 Leu-->Arg) This is a rare hemoglobin variant, which until now has been reported only once. The clinical course of the 12-year-old boy was characterized by severe hemolytic anemia leading to splenectomy and cholecystectomy at the of 3.5 years. The priapism occurred 8 years after splenectomy, during a hemolytic febrile episode and required aspiration of the corpora cavernosa. This report raises the question of the benefit of splenectomy in patients suffering from a chronic hemolytic anemia such as that due to an unstable hemoglobin. This treatment lowers the frequency and the severity of acute hemolytic attacks, but several cases of vascular complications have been reported after splenectomy.
Databáze: OpenAIRE
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