Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)
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 a...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2019/3146965 |
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| author | Ethan Burns Kartik Anand Gonzalo Acosta Malcolm Irani Betty Chung Abhishek Maiti Ibrahim Ibrahim Lawrence Rice |
| author_facet | Ethan Burns Kartik Anand Gonzalo Acosta Malcolm Irani Betty Chung Abhishek Maiti Ibrahim Ibrahim Lawrence Rice |
| author_sort | Ethan Burns |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-c70cf100456a4197aca2c76098b367d8 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-c70cf100456a4197aca2c76098b367d82025-02-03T01:12:42ZengWileyCase Reports in Hematology2090-65602090-65792019-01-01201910.1155/2019/31469653146965Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)Ethan Burns0Kartik Anand1Gonzalo Acosta2Malcolm Irani3Betty Chung4Abhishek Maiti5Ibrahim Ibrahim6Lawrence Rice7Houston Methodist Hospital, Department of Medicine, 6550 Fannin St, Houston, TX 77030, USAHouston Methodist Hospital, Department of Medicine, 6550 Fannin St, Houston, TX 77030, USAHouston Methodist Hospital, Department of Medicine, 6550 Fannin St, Houston, TX 77030, USAHouston Methodist Hospital, Department of Medicine, 6550 Fannin St, Houston, TX 77030, USAHouston Methodist Hospital, Department of Pathology and Genomic Medicine, 6550 Fannin St, Houston, TX 77030, USAThe University of Texas MD Anderson Cancer Center, Division of Cancer Medicine, 1515 Holcombe Blvd, Houston, Texas 77030, USAUniversity of Texas Southwestern, Department of Internal Medicine, Division of Hematology/Oncology, 5323 Harry Hines Blvd, Dallas, TX 75390, USAHouston Methodist Hospital, Department of Medicine, 6550 Fannin St, Houston, TX 77030, USAAutosplenectomy (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.http://dx.doi.org/10.1155/2019/3146965 |
| spellingShingle | Ethan Burns Kartik Anand Gonzalo Acosta Malcolm Irani Betty Chung Abhishek Maiti Ibrahim Ibrahim Lawrence Rice Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH) Case Reports in Hematology |
| title | Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH) |
| title_full | Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH) |
| title_fullStr | Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH) |
| title_full_unstemmed | Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH) |
| title_short | Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH) |
| title_sort | autosplenectomy in a patient with paroxysmal nocturnal hemoglobinuria pnh |
| url | http://dx.doi.org/10.1155/2019/3146965 |
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