Even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long-term sustained intolerant fatigue
Fatigue is one of the most common symptoms associated with paroxysmal nocturnal hemoglobinuria (PNH), a rare acquired disorder of hematopoietic stem cells. While it directly impairs lifestyle leading to poor quality of life (QOL), it is not well recognized that fatigue could not depend on the diseas...
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| Format: | Article |
| Language: | English |
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Elsevier
2020-01-01
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| Series: | Leukemia Research Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213048920300303 |
| _version_ | 1818363919919480832 |
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| author | Kazuya Sakai |
| author_facet | Kazuya Sakai |
| author_sort | Kazuya Sakai |
| collection | DOAJ |
| description | Fatigue is one of the most common symptoms associated with paroxysmal nocturnal hemoglobinuria (PNH), a rare acquired disorder of hematopoietic stem cells. While it directly impairs lifestyle leading to poor quality of life (QOL), it is not well recognized that fatigue could not depend on the disease activity or percentage of glycosylphosphatidylinositol-deficient granulocyte. We describe the case of a 78-year-old woman, with mild hemolysis and a 20-year history of severe sustained fatigue, whose QOL drastically improved with eculizumab followed by ravulizumab. We also used the novel aplastic anemia and PNH specific QOL tool to evaluate multiple statuses of the patient. |
| first_indexed | 2024-12-13T21:56:08Z |
| format | Article |
| id | doaj.art-19c8abaa462c47279b3ee928661300a5 |
| institution | Directory Open Access Journal |
| issn | 2213-0489 |
| language | English |
| last_indexed | 2024-12-13T21:56:08Z |
| publishDate | 2020-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Leukemia Research Reports |
| spelling | doaj.art-19c8abaa462c47279b3ee928661300a52022-12-21T23:30:08ZengElsevierLeukemia Research Reports2213-04892020-01-0114100224Even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long-term sustained intolerant fatigueKazuya Sakai0Department of Hematology, Nara City Hospital, 50-1 Higashikidera-Cho 1 Chome, Nara city, Nara 630-8305, JapanFatigue is one of the most common symptoms associated with paroxysmal nocturnal hemoglobinuria (PNH), a rare acquired disorder of hematopoietic stem cells. While it directly impairs lifestyle leading to poor quality of life (QOL), it is not well recognized that fatigue could not depend on the disease activity or percentage of glycosylphosphatidylinositol-deficient granulocyte. We describe the case of a 78-year-old woman, with mild hemolysis and a 20-year history of severe sustained fatigue, whose QOL drastically improved with eculizumab followed by ravulizumab. We also used the novel aplastic anemia and PNH specific QOL tool to evaluate multiple statuses of the patient.http://www.sciencedirect.com/science/article/pii/S2213048920300303Paroxysmal nocturnal hemoglobinuriaQuality of lifeSustained fatigueEculizumab Ravulizumab |
| spellingShingle | Kazuya Sakai Even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long-term sustained intolerant fatigue Leukemia Research Reports Paroxysmal nocturnal hemoglobinuria Quality of life Sustained fatigue Eculizumab Ravulizumab |
| title | Even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long-term sustained intolerant fatigue |
| title_full | Even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long-term sustained intolerant fatigue |
| title_fullStr | Even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long-term sustained intolerant fatigue |
| title_full_unstemmed | Even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long-term sustained intolerant fatigue |
| title_short | Even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long-term sustained intolerant fatigue |
| title_sort | even mild hemolysis in paroxysmal nocturnal hemoglobinuria could severely compromise the quality of life due to long term sustained intolerant fatigue |
| topic | Paroxysmal nocturnal hemoglobinuria Quality of life Sustained fatigue Eculizumab Ravulizumab |
| url | http://www.sciencedirect.com/science/article/pii/S2213048920300303 |
| work_keys_str_mv | AT kazuyasakai evenmildhemolysisinparoxysmalnocturnalhemoglobinuriacouldseverelycompromisethequalityoflifeduetolongtermsustainedintolerantfatigue |