Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda. (31st January 2019)

• Record Type: Journal Article
• Authors: Lumori, Boniface Amanee Elias; Muyanja, Daniel
• Other Names: Kyrtsonis Marie-Christine Academic Editor.
• Is Part Of: Case reports in hematology. Volume 2019(2019)
• Publication Date: 2019-01-31
• Subjects: Hematology -- Periodicals; Hematology -- Case studies -- Periodicals; Blood -- Diseases -- Periodicals; Hematology; Hematologic Diseases; Blood -- Diseases; Hematology; Electronic journals; Periodicals; Case studies; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 616.15
• DOI: 10.1155/2019/7897509
• Languages: English
• ISSNs: 2090-6560

Abstract

Abstract : 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.