June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Treatment of Adult Orbital Xanthogranuloma with Intravenous Rituximab
Author Affiliations & Notes
  • Michelle Wilson Latting
    Ophthalmology, Wills Eye Hospital, Philadelphia, Pennsylvania, United States
  • Charles Kim
    Oculoplastic and Orbital Surgery, Wills Eye Hospital, Philadelphia, Pennsylvania, United States
  • Mary Stefanyszyn
    Oculoplastic and Orbital Surgery, Wills Eye Hospital, Philadelphia, Pennsylvania, United States
  • Footnotes
    Commercial Relationships   Michelle Latting, None; Charles Kim, None; Mary Stefanyszyn, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 5150. doi:
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      Michelle Wilson Latting, Charles Kim, Mary Stefanyszyn; Treatment of Adult Orbital Xanthogranuloma with Intravenous Rituximab. Invest. Ophthalmol. Vis. Sci. 2017;58(8):5150.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To describe the clinical and histopathologic response of adult orbital xanthogranuloma treated with intravenous rituximab.

Methods : A 42 year-old male with adult orbital xanthogranuloma refractory to multiple treatment modalities received intravenous rituximab infusions for treatment of his xanthogranulomatous disease. Biopsy of orbital tissue was obtained prior to rituximab therapy and after the conclusion of therapy.

Results : The infiltrate was debulked in the left orbit and the pathologic specimen revealed histiocytes with foamy vacuolated cytoplasm, scattered Touton giant cells, and foci of well circumscribed reactive lymphoid follicles. Immunohistochemical markers disclosed a mixture of CD20 positive B-lymphocytes as well as CD3 positive and CD5 positive T-lymphocytes. BCL-2 was negative consistent with a reactive process. The disease progressed in the right orbit and the patient was treated with six infusions of intravenous rituximab. Following treatment, the right orbit was debulked and biopsied. The pathologic specimen contained adipose tissue and occasional lymphocytes. The xanthogranulomatous infiltrate and lymphoid follicles were resolved. After rituximab therapy, bilateral reduction in orbital soft tissue fullness was noted with no further progression or recurrence at 20 months.

Conclusions : Adult orbital xanthogranuloma is a rare disease that has been a treatment challenge. The response to surgical debulking and medical treatment modalities including corticosteroids and antimetabolites has been variable with progression or recurrences common. Rituximab, a monoclonal antibody directed against CD20 antigens found on B cells, is an alterative treatment that may result in disease stability or clinical remission.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

 

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