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CMV Retinitis in Wiskott Aldrich Syndrome
Jia Luo1, Jie Peng1, Pei-Quan Zhao1
1Xinhua Hospital, Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Ocular Immunology and Inflammation
|December 8, 2021
Summary
Hematopoietic stem cell transplantation (HSCT) is a curative treatment for Wiskott-Aldrich syndrome (WAS). Patients with WAS require regular eye exams, especially before HSCT and for 3.5 months after, to manage cytomegalovirus retinitis (CMVR).
Area of Science:
- Ophthalmology
- Hematology
- Immunology
Background:
- Wiskott-Aldrich syndrome (WAS) is a serious X-linked immune deficiency.
- Hematopoietic stem cell transplantation (HSCT) is the primary curative treatment for WAS.
- Cytomegalovirus retinitis (CMVR) is a potential complication causing vision loss in WAS patients.
Purpose of the Study:
- To investigate the progression and prognosis of cytomegalovirus retinitis (CMVR) in patients with Wiskott-Aldrich syndrome (WAS).
- To evaluate the impact of HSCT on CMVR development and outcomes in WAS patients.
Main Methods:
- Retrospective case series of ten WAS patients with CMVR.
- Data collected from patients referred to Xinhua Hospital's Ophthalmology Department (June 2018 - February 2021).
- Analysis of CMVR occurrence and progression before and after HSCT.
Main Results:
- Five WAS patients were diagnosed with CMVR before HSCT (median age 10.5 months).
- Eight WAS patients developed CMVR post-HSCT (median 3.5 months interval).
Conclusions:
- Regular fundus examinations are crucial for early CMVR detection in WAS patients.
- Prompt treatment of CMVR is essential before HSCT and for approximately 3.5 months post-HSCT.
- Monitoring is vital until full immune reconstitution following HSCT.
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