CMV Retinitis in Wiskott Aldrich Syndrome

ABSTRACT

Purpose: Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disease whose optimal curative treatment is hematopoietic stem cell transplantation (HSCT). Patients with WAS may suffer from cytomegalovirus retinitis (CMVR) which can cause vision loss. This study is to report the progression and prognosis of patients with WAS and CMVR. Methods: A retrospective case series of ten patients with WAS and CMVR before and after HSCT who were referred to the Ophthalmology Department of Xinhua Hospital from June 2018 to February 2021. Progression and prognosis were recorded. Results: Five patients were diagnosed with CMVR before receiving HSCT at a median age of 10.5 months (range: 4–23 months). Eight patients developed CMVR post-transplantation with a median interval from HSCT of 3.5 months (range: 1–9 months). Conclusion: Regular fundus examinations and prompt treatments in patients with WAS are therefore crucial before they receiving HSCT or approximately 3.5 months after HSCT until complete reconstitution of immune function.

KEYWORDS:

• Wiskott-Aldrich syndrome
• cytomegalovirus retinitis
• hematopoietic stem cell transplantation

Acknowledgments

The authors would like to thank all the patients and their families for their participation.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

This research was supported by the National Natural Science Foundation of China (No. 81770963, 81770964)