Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda
Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare disorder of the hematopoietic stem cells which is often underdiagnosed. Its incidence is about 5 cases per million inhabitants in a year, and currently, there are only 1610 patients in the International PNH Registry. In this repo...
Saved in:
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2019-01-01
|
| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2019/7897509 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832565533371793408 |
|---|---|
| author | Boniface Amanee Elias Lumori Daniel Muyanja |
| author_facet | Boniface Amanee Elias Lumori Daniel Muyanja |
| author_sort | Boniface Amanee Elias Lumori |
| collection | DOAJ |
| description | Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare disorder of the hematopoietic stem cells which is often underdiagnosed. Its incidence is about 5 cases per million inhabitants in a year, and currently, there are only 1610 patients in the International PNH Registry. In this report, we describe a case of PNH in southwestern Uganda. Case Presentation. A 34-year-old man, subsistence farmer, with a history of multiple prior presentations with anemia, jaundice, and dark-colored urine requiring blood transfusions presented to us again in July 2018 with a week’s history of palpitations, dizziness, and dark-colored urine. Investigations done suggested a direct antiglobulin test- (DAT-) negative hemolytic anemia, and subsequently, flow cytometry showed a large clone of PNH. He received many blood transfusions and hematinics on several occasions during the course of his admissions. Conclusions. Our report showed diagnostic and treatment challenges of PNH in health resource-limited setting. |
| format | Article |
| id | doaj-art-c8049ebcc1334c4e9a9a21cdd82de6ad |
| 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-c8049ebcc1334c4e9a9a21cdd82de6ad2025-02-03T01:07:29ZengWileyCase Reports in Hematology2090-65602090-65792019-01-01201910.1155/2019/78975097897509Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in UgandaBoniface Amanee Elias Lumori0Daniel Muyanja1Department of Internal Medicine, Mbarara University of Science and Technology (MUST), Mbarara, UgandaDepartment of Internal Medicine, Mbarara University of Science and Technology (MUST), Mbarara, UgandaIntroduction. Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare disorder of the hematopoietic stem cells which is often underdiagnosed. Its incidence is about 5 cases per million inhabitants in a year, and currently, there are only 1610 patients in the International PNH Registry. In this report, we describe a case of PNH in southwestern Uganda. Case Presentation. A 34-year-old man, subsistence farmer, with a history of multiple prior presentations with anemia, jaundice, and dark-colored urine requiring blood transfusions presented to us again in July 2018 with a week’s history of palpitations, dizziness, and dark-colored urine. Investigations done suggested a direct antiglobulin test- (DAT-) negative hemolytic anemia, and subsequently, flow cytometry showed a large clone of PNH. He received many blood transfusions and hematinics on several occasions during the course of his admissions. Conclusions. Our report showed diagnostic and treatment challenges of PNH in health resource-limited setting.http://dx.doi.org/10.1155/2019/7897509 |
| spellingShingle | Boniface Amanee Elias Lumori Daniel Muyanja Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda Case Reports in Hematology |
| title | Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda |
| title_full | Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda |
| title_fullStr | Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda |
| title_full_unstemmed | Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda |
| title_short | Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda |
| title_sort | diagnostic and treatment challenges of paroxysmal nocturnal hemoglobinuria in uganda |
| url | http://dx.doi.org/10.1155/2019/7897509 |
| work_keys_str_mv | AT bonifaceamaneeeliaslumori diagnosticandtreatmentchallengesofparoxysmalnocturnalhemoglobinuriainuganda AT danielmuyanja diagnosticandtreatmentchallengesofparoxysmalnocturnalhemoglobinuriainuganda |