Primary orbital lymphomatoid granulomatosis

Araki, Fumiyuki; Mimura, Tatsuya; Fukuoka, Shima; Tsuji, Hideki; Izutsu, Koji; Yamamoto, H.; Takazawa, Yutaka; Kojima, Tetsu

doi:10.1136/bjo.2008.140913

Cited by 3 publications

(2 citation statements)

References 5 publications

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“… 6 These include conjunctival, pupillary, choroidal, retinal, optic nerve, and orbital (including lacrimal gland) involvement. 6 7 8 All but one of these cases were described in association with pulmonary or other systemic involvement. The only other report of primary orbital involvement occurred in an otherwise healthy 54-year-old man.…”

Section: Case Reportmentioning

confidence: 99%

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Primary orbital lymphomatoid granulomatosis in a 1-year-old child

Aboobaker

Tinley

2015

Middle East Afr J Ophthalmol

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Section: Case Reportmentioning

confidence: 99%

“…The only other report of primary orbital involvement occurred in an otherwise healthy 54-year-old man. 8 The disease is most common after the fifth decade and is very rare is children. There are three previously reported cases of pediatric ophthalmic LyG, in children of ages 7, 8, and 13.…”

Section: Case Reportmentioning

confidence: 99%

Primary orbital lymphomatoid granulomatosis in a 1-year-old child

Aboobaker

Tinley

2015

Middle East Afr J Ophthalmol

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Orbital lymphomatoid granulomatosis – a rare cause of proptosis

et al. 2014

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A 1-year-old girl with unilateral proptosis was found to have primary orbital lymphomatoid granulomatosis - a condition rarely occurring in children. This multisystem angiocentric, angiodestructive, lymphoproliferative disease typically involves the lungs, with ocular involvement being extremely uncommon. Our case serves to illustrate the imaging findings of this unusual condition and highlight a rare cause of proptosis.

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Lymphomatoid Granulomatosis

Bollerslev¹,

Kruse²,

Larsen³

2016

Diagnostic Pathology: Neuropathology

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Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease. The atypical lymphoid cells directly accumulate within affected tissues and clinically present in the form of infiltrative lesions. It is usually a progressive disorder that virtually always involves the lung and characteristically presents as bilateral pulmonary nodules. Other commonly affected organ systems include the skin, central nervous system, and kidneys. The rareness of LYG in conjunction with its nonspecific presentation contributes to delays in diagnosis in many situations. Pathologically, it is characterized by the presence of an angiocentric and angiodestructive accumulation of varying numbers of T cells with varying numbers of atypical clonal EBV-positive B cells in a polymorphous inflammatory background. It can be subclassified using a grading system based on the number of EBV-positive large B-cell malignant cells, which is critical in selecting appropriate management strategies. Lower-grade LYG occasionally undergoes spontaneous remission and is best managed with strategies designed to enhance the host's underlying immune system, whereas high-grade LYG is best managed by combination chemoimmunotherapy but has inferior outcomes. Lymphomatoid granulomatosis can lead to progressive pulmonary failure, central nervous system disease, or progression to overt EBV-positive lymphoma without appropriate recognition and management. Improvements in the modern understanding of the biology of LYG, particularly the precise role of EBV in its pathogenesis, offer promise in the development of improved management strategies.

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Primary orbital lymphomatoid granulomatosis

Cited by 3 publications

References 5 publications

Primary orbital lymphomatoid granulomatosis in a 1-year-old child

Primary orbital lymphomatoid granulomatosis in a 1-year-old child

Orbital lymphomatoid granulomatosis – a rare cause of proptosis

Lymphomatoid Granulomatosis

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