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Primary Orbital Lymphomatoid Granulomatosis in a 1-Year-Old Child.

Aboobaker S, Tinley C - Middle East Afr J Ophthalmol (2015 Oct-Dec)

Bottom Line: Lymphomatoid granulomatosis (LyG) is a rare, angiocentric, angiodestructive, B-cell lymphoproliferative disease, presenting with pulmonary involvement in more than 80% of cases.We describe a case of primary orbital LyG in a 1-year-old child.

View Article: PubMed Central - PubMed

Affiliation: Division of Ophthalmology, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.

ABSTRACT
Lymphomatoid granulomatosis (LyG) is a rare, angiocentric, angiodestructive, B-cell lymphoproliferative disease, presenting with pulmonary involvement in more than 80% of cases. We describe a case of primary orbital LyG in a 1-year-old child.

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Angiocentric and angiodestructive tumor cells occluding a blood vessel
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Figure 3: Angiocentric and angiodestructive tumor cells occluding a blood vessel

Mentions: A transnasal biopsy led to the provisional diagnosis of a diffuse, large B-cell lymphoma. Induction therapy consisting of cyclophosphamide, vincristine, and prednisone was commenced, according to the French Society of Pediatric Ophthalmology LMB89 protocol for large B-cell lymphoma.1 Later immunohistochemistry showed more than 50 Epstein-Barr virus (EBV) positive cells per high power field (hpf), CD 20 positive B-cells in a background of CD 3 positive T-cells, and tumor cells in and around blood vessels [Figure 3]. These findings are consistent with the diagnosis of LyG Grade III. Ganciclovir was therefore added to her treatment, in addition to methotrexate, cytarabine, and etoposide.2 Within a week, there was a marked reduction in tumor size on this regime.


Primary Orbital Lymphomatoid Granulomatosis in a 1-Year-Old Child.

Aboobaker S, Tinley C - Middle East Afr J Ophthalmol (2015 Oct-Dec)

Angiocentric and angiodestructive tumor cells occluding a blood vessel
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4660543&req=5

Figure 3: Angiocentric and angiodestructive tumor cells occluding a blood vessel
Mentions: A transnasal biopsy led to the provisional diagnosis of a diffuse, large B-cell lymphoma. Induction therapy consisting of cyclophosphamide, vincristine, and prednisone was commenced, according to the French Society of Pediatric Ophthalmology LMB89 protocol for large B-cell lymphoma.1 Later immunohistochemistry showed more than 50 Epstein-Barr virus (EBV) positive cells per high power field (hpf), CD 20 positive B-cells in a background of CD 3 positive T-cells, and tumor cells in and around blood vessels [Figure 3]. These findings are consistent with the diagnosis of LyG Grade III. Ganciclovir was therefore added to her treatment, in addition to methotrexate, cytarabine, and etoposide.2 Within a week, there was a marked reduction in tumor size on this regime.

Bottom Line: Lymphomatoid granulomatosis (LyG) is a rare, angiocentric, angiodestructive, B-cell lymphoproliferative disease, presenting with pulmonary involvement in more than 80% of cases.We describe a case of primary orbital LyG in a 1-year-old child.

View Article: PubMed Central - PubMed

Affiliation: Division of Ophthalmology, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.

ABSTRACT
Lymphomatoid granulomatosis (LyG) is a rare, angiocentric, angiodestructive, B-cell lymphoproliferative disease, presenting with pulmonary involvement in more than 80% of cases. We describe a case of primary orbital LyG in a 1-year-old child.

Show MeSH
Related in: MedlinePlus