Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)
Joint Authors
Rice, Lawrence
Burns, Ethan
Acosta, Gonzalo
Anand, Kartik
Irani, Malcolm
Chung, Betty
Maiti, Abhishek
Ibrahim, Ibrahim
Source
Issue
Vol. 2019, Issue 2019 (31 Dec. 2019), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2019-02-12
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Autosplenectomy (AS) is a known complication of diseases such as sickle cell anemia, celiac disease, and inflammatory bowel disease.
We report the first known case of AS due to paroxysmal nocturnal hemoglobinuria (PNH).
A 24-year-old Caucasian male had evidence of hemolytic anemia at the age of 14 and was diagnosed with PNH at the age of 16.
He had recurrent episodes of sepsis due to dialysis line infections from poor hygiene, and blood cultures had been positive for multiple organisms including Staphylococcus aureus, Enterococcus faecalis, and Streptococcus pneumoniae.
The patient’s peripheral blood smears since the age of 14 years demonstrated Howell–Jolly bodies in conjunction with thrombocytopenia and hemolytic anemia, but abdominal ultrasonography reported a normal appearing spleen.
The patient presented with septicemia two years after starting eculizumab, and his peripheral blood smear showed extensive Howell–Jolly bodies, Pappenheimer bodies, acanthocytes, and target cells.
Splenic ultrasonography demonstrated an atrophic spleen with multifocal scarring, and absent splenic uptake of liver-spleen scintigraphy, consistent with AS.
Clinicians should remain vigilant of the potential sequelae of PNH and consider the possibility of the development of AS.
American Psychological Association (APA)
Burns, Ethan& Anand, Kartik& Acosta, Gonzalo& Irani, Malcolm& Chung, Betty& Maiti, Abhishek…[et al.]. 2019. Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH). Case Reports in Hematology،Vol. 2019, no. 2019, pp.1-5.
https://search.emarefa.net/detail/BIM-1136494
Modern Language Association (MLA)
Burns, Ethan…[et al.]. Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH). Case Reports in Hematology No. 2019 (2019), pp.1-5.
https://search.emarefa.net/detail/BIM-1136494
American Medical Association (AMA)
Burns, Ethan& Anand, Kartik& Acosta, Gonzalo& Irani, Malcolm& Chung, Betty& Maiti, Abhishek…[et al.]. Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH). Case Reports in Hematology. 2019. Vol. 2019, no. 2019, pp.1-5.
https://search.emarefa.net/detail/BIM-1136494
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1136494