A Rapidly Fatal Case of T-Cell Lymphoma Presenting as Idiopathic Orbital Inflammation

Papalkar, Daya; Sharma, Shanel; Francis, Ian C.; Downie, J. A.; Thanakrishnan, Govindasamy; Hughes, Louise

doi:10.1080/01676830490916064

Cited by 22 publications

(10 citation statements)

References 8 publications

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“…Termote et al 19 reported a case series comprising three patients with periorbital involvement from NKTL who all developed painless eyelid swelling, which, along with a history of chronic sinusitis with nasal congestion, masqueraded as periorbital cellulitis. The diagnosis was established from biopsies of the orbital mass, and in one case of the cervical lymphadenopathy 17 . Similarly, 50% and 16.7% of the patients in group 1 presented with symptoms mimicking orbital cellulitis and pseudotumor, respectively.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma involving the ocular adnexa

et al. 2018

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Our series is to our knowledge the largest cohort study on NKTL reported to date and demonstrates that ocular adnexal NKTL is a rare but seriously fatal disease. It is characterised by acute inflammatory signs as present in as many as two-thirds of our patients in this series. It should be considered as a differential diagnosis in patients presenting with rapidly progressing proptosis and diagnosed promptly for optimal management.

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Section: Discussionmentioning

confidence: 99%

Clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma involving the ocular adnexa

et al. 2018

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“…Therefore, these composite diagnostic processes confirming the BIOMED-2 PCR assay results can be limitedly useful in diagnosing equivocal lymphomas that are caused by previous use of corticosteroids 13 . This diagnostic attempt is of clinical value because it may reduce the risk of under treatment of undiagnosed OAL 25 …”

Section: Discussionmentioning

confidence: 99%

Two cases of primary ocular adnexal lymphomas diagnosed after pre-biopsy corticosteroid treatment using polymerase chain reaction-based gene rearrangement analysis

Kitahara

Imamura

Ohta

et al. 2019

American Journal of Ophthalmology Case Reports

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Purpose To report the limited usefulness of polymerase chain reaction (PCR)-based immunoglobulin (Ig) and T-cell receptor (TCR) gene rearrangement analysis in diagnosing primary ocular adnexal lymphomas (OAL) treated with corticosteroids before biopsy. Observations This was a case series of two patients: a 47-year-old woman and a 43-year-old man, who both presented with impaired visual acuity and ophthalmoplegia of the involved eyes. Both patients had previously received non-diagnostic biopsy and had been subsequently treated with corticosteroids. The visual acuity and ophthalmoplegia progressively worsened after a variable duration of remission. Ocular magnetic resonance imaging revealed gadolinium-enhancing intra- and extraconal lesions. Systemic evaluations did not reveal any other lesions outside of the orbit. Differential diagnoses were lymphoproliferative disorders, including undiagnosed primary OALs, and idiopathic ocular inflammation. Both patients were exposed to repeated biopsies. The biopsied tissue demonstrated marked lymphocytolysis due to corticosteroid usage; therefore, histology and immunophenotype were non-diagnostic. EuroClonality/BIOMED-2 PCR-based gene rearrangement analyses detected genetic clonalities of Ig and TCR and suggested diagnoses of primary OALs of B-cell and T-cell origins, respectively. An OAL of B-cell origin was treated with radiotherapy; an OAL of a rare T-cell origin was treated with high-dose methotrexate-based chemotherapy and adjuvant radiotherapy. Both patients remained progression free for more than 36 months. Conclusions and importance PCR-based gene rearrangement analysis can be of limited usefulness in suggesting a diagnosis of primary OAL in patients receiving pre-biopsy corticosteroid treatment. Identification of genetic clonality is of clinical importance to provide treatment options for undiagnosed OALs.

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“…There is a report of a 41year-old woman, although Caucasian, who was initially diagnosed with idiopathic orbital inflammation and later died of widespread ENKTL. 9 Another report demonstrated that NKTL comprises less than 1% of OAL in Western countries. 5 However, NKTL is often a fatal disease, especially when the diagnosis is delayed, as in our case, and has been shown to be more prevalent in East Asia than in Western countries.…”

Section: To the Editormentioning

confidence: 99%

Orbital Inflammatory Lesion as an Initial Manifestation of Systemic Nasal Type NK/T-Cell Lymphoma

Kawakami¹,

Ito²,

Yazaki³

et al. 2012

J Clin Exp Hematopathol

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A Rapidly Fatal Case of T-Cell Lymphoma Presenting as Idiopathic Orbital Inflammation

Cited by 22 publications

References 8 publications

Clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma involving the ocular adnexa

Clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma involving the ocular adnexa

Two cases of primary ocular adnexal lymphomas diagnosed after pre-biopsy corticosteroid treatment using polymerase chain reaction-based gene rearrangement analysis

Orbital Inflammatory Lesion as an Initial Manifestation of Systemic Nasal Type NK/T-Cell Lymphoma

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