Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort study

Introduction: Sickle cell anemia (SCA) is one of the most common genetic diseases worldwide. Patients with SCA present with varied clinical features and complications that may affect many organs in the human body. There are few treatment options for SCA, and patient responses vary; the only curative...

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Main Authors: Abdulrahman Musaad Alhumaid, Abdulmalek Suliman Aleidi, Abdullelah Saleh Alfakhri, Naif Khalil Alosaimi, Yosra Z Ali, Mohsen Saadi Alzahrani
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2018-01-01
Series:Journal of Applied Hematology
Subjects:
Online Access:http://www.jahjournal.org/article.asp?issn=1658-5127;year=2018;volume=9;issue=1;spage=22;epage=28;aulast=Alhumaid
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spelling doaj-09bdbefd069f4056892b6c48b6b1b6e12020-11-24T20:44:14ZengWolters Kluwer Medknow PublicationsJournal of Applied Hematology1658-51272018-01-0191222810.4103/joah.joah_48_17Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort studyAbdulrahman Musaad AlhumaidAbdulmalek Suliman AleidiAbdullelah Saleh AlfakhriNaif Khalil AlosaimiYosra Z AliMohsen Saadi AlzahraniIntroduction: Sickle cell anemia (SCA) is one of the most common genetic diseases worldwide. Patients with SCA present with varied clinical features and complications that may affect many organs in the human body. There are few treatment options for SCA, and patient responses vary; the only curative therapy is a stem cell transplant or gene therapy. We seek to study the clinical features and treatment options for patients with sickle cell disease treated in King Abdulaziz Medical City, Riyadh. Methods: This study was a retrospective cohort study of all adult SCA patients who were admitted to our institution during the period from 1983 to 2016. Data were entered into Excel spreadsheets and managed with SPSS. Chi-square test was used to compare responses to therapy and in patients with different presentations. Results: A total of 106 patients with sickle cell disease were included in this study. The percentages with respect to hospital admissions per year were as follows: 34.9% (37 patients) were never admitted, 26.4% (28 patients) were admitted fewer than 2 times, 24.5% (26 patients) were admitted 3–5 times, and 13.2% (14 patients) were admitted more than 5 times. The number of complications was used to measure the severity of the disease. The disease severity was higher in males than in females (67.3% vs. 32.7%, respectively; P = 0.018). The disease severity was higher in those who were born in the Western and Southwestern areas than in other areas (84.2% vs. 15.8%, respectively; P = 0.007). Hydroxyurea significantly reduced the severity of SCA with a P = 0.002. Conclusion: Our study showed that vaso-occlusive crisis was the most common complication and indication for hospital admission. Treatment with hydroxyurea led to a significant reduction in the number of hospital admissions.http://www.jahjournal.org/article.asp?issn=1658-5127;year=2018;volume=9;issue=1;spage=22;epage=28;aulast=AlhumaidAnemiacomplicationhemoglobinhemoglobin SShospitalizationsickle cellsickle cell disease
collection DOAJ
language English
format Article
sources DOAJ
author Abdulrahman Musaad Alhumaid
Abdulmalek Suliman Aleidi
Abdullelah Saleh Alfakhri
Naif Khalil Alosaimi
Yosra Z Ali
Mohsen Saadi Alzahrani
spellingShingle Abdulrahman Musaad Alhumaid
Abdulmalek Suliman Aleidi
Abdullelah Saleh Alfakhri
Naif Khalil Alosaimi
Yosra Z Ali
Mohsen Saadi Alzahrani
Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort study
Journal of Applied Hematology
Anemia
complication
hemoglobin
hemoglobin SS
hospitalization
sickle cell
sickle cell disease
author_facet Abdulrahman Musaad Alhumaid
Abdulmalek Suliman Aleidi
Abdullelah Saleh Alfakhri
Naif Khalil Alosaimi
Yosra Z Ali
Mohsen Saadi Alzahrani
author_sort Abdulrahman Musaad Alhumaid
title Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort study
title_short Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort study
title_full Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort study
title_fullStr Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort study
title_full_unstemmed Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort study
title_sort clinical features and outcome of sickle cell anemia in a tertiary center: a retrospective cohort study
publisher Wolters Kluwer Medknow Publications
series Journal of Applied Hematology
issn 1658-5127
publishDate 2018-01-01
description Introduction: Sickle cell anemia (SCA) is one of the most common genetic diseases worldwide. Patients with SCA present with varied clinical features and complications that may affect many organs in the human body. There are few treatment options for SCA, and patient responses vary; the only curative therapy is a stem cell transplant or gene therapy. We seek to study the clinical features and treatment options for patients with sickle cell disease treated in King Abdulaziz Medical City, Riyadh. Methods: This study was a retrospective cohort study of all adult SCA patients who were admitted to our institution during the period from 1983 to 2016. Data were entered into Excel spreadsheets and managed with SPSS. Chi-square test was used to compare responses to therapy and in patients with different presentations. Results: A total of 106 patients with sickle cell disease were included in this study. The percentages with respect to hospital admissions per year were as follows: 34.9% (37 patients) were never admitted, 26.4% (28 patients) were admitted fewer than 2 times, 24.5% (26 patients) were admitted 3–5 times, and 13.2% (14 patients) were admitted more than 5 times. The number of complications was used to measure the severity of the disease. The disease severity was higher in males than in females (67.3% vs. 32.7%, respectively; P = 0.018). The disease severity was higher in those who were born in the Western and Southwestern areas than in other areas (84.2% vs. 15.8%, respectively; P = 0.007). Hydroxyurea significantly reduced the severity of SCA with a P = 0.002. Conclusion: Our study showed that vaso-occlusive crisis was the most common complication and indication for hospital admission. Treatment with hydroxyurea led to a significant reduction in the number of hospital admissions.
topic Anemia
complication
hemoglobin
hemoglobin SS
hospitalization
sickle cell
sickle cell disease
url http://www.jahjournal.org/article.asp?issn=1658-5127;year=2018;volume=9;issue=1;spage=22;epage=28;aulast=Alhumaid
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