Nasal NK/T cell lymphoma with severe facial disfigurement in a 37-year-old male

Li, Z.; Liu, C.; Huang, Xiaowei; Gao, Zifen

doi:10.1016/j.ijom.2012.06.021

Cited by 3 publications

(8 citation statements)

References 16 publications

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“…We searched the PubMed database using the search terms “extranodal NK T-cell lymphoma nasal type AND case report.” We found 56 case reports with the diagnosis of ENKL written in English. Among them, the ANCA titers were measured in six reports ( 17 - 22 ), only one of which report were positive for ANCAs ( 21 ). Furthermore, the infiltration of neutrophils was observed in only three reports ( 23 - 25 ).…”

Section: Discussionmentioning

confidence: 99%

Extranodal NK/T-cell Lymphoma, Nasal Type Accompanied by PR3-ANCA-associated Glomerulonephritis

et al. 2017

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A 62-year-old man exhibiting nasal obstruction and glomerulonephritis with proteinase 3-antineutrophil cytoplasmic antibodies (PR3-ANCAs) was diagnosed with extranodal NK/T-cell lymphoma, nasal type (ENKL) with infiltration of neutrophils with apoptosis. Chemoradiotherapy reduced the tumor, improved the renal function, and decreased the PR3-ANCA levels. ANCA-positivity is observed in immunoinsufficient diseases, in which neutrophils lead to apoptosis and translocate intracellular granules, such as PR3, to the cell surface, triggering the production of ANCAs. In our case, the PR3-ANCA production was derived from the expression of PR3 on the cell surface of apoptotic neutrophils. This is the first report on ENKL describing the mechanism of ANCA development.

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Section: Discussionmentioning

confidence: 99%

Extranodal NK/T-cell Lymphoma, Nasal Type Accompanied by PR3-ANCA-associated Glomerulonephritis

et al. 2017

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“…In a case that we reported in 2012 ( 16), Mucor irregularis was identified in ENKTL-NT (20). The fungus eroded through his sinuses and hard palate, resulting in destructive changes in mid-face, maxilla, mandible, and partial orbit with systemic manifestations of fever, weight loss, hepatosplenomegaly, and anemia.…”

Section: Fungi and Enktl-ntmentioning

confidence: 96%

“…Besides, ROCM is featured by the detection of fungi in the tissue (16,19,69), while ENKTL-NT is typically associated with NK/T cells, hyperplasia, and angiodestruction (3,110,13,14,33,111). In some cases, both features were present in patients with MNS, which made the diagnosis rather confused (16,(20)(21)(22)27).…”

Section: Pathological Characteristicsmentioning

confidence: 99%

“…Necrosis, acute or progressive, focal or diffuse, commonly ischemic with inclusive vasculitis and giant cell infiltration, is the predominant feature of ENKTL-NT that may follow thrombosis or embolism (12,16,13,19,20,112,113). Sometimes, necrosis occurs suddenly like an acute vascular event (3,9,16,114,115).…”

Section: Necrosis Inflammation and Granulationmentioning

confidence: 99%

“…ROCM is featured by the detection of fungi in the tissue, while ENKTL-NT is typically associated with NK/T-cell infiltration, hyperplasia, and angiocentric characteristics. Diagnosis is often confusing when both these features are present in the tissues of patients with facial destruction (16,20,21).…”

Section: Introductionmentioning

confidence: 99%

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Rhino-Orbital-Cerebral Mycosis and Extranodal Natural Killer or/and T-Cell Lymphoma, Nasal Type

Lun

2022

Front. Med.

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BackgroundExtranodal natural killer/T-cell lymphoma, nasal type is a syndrome of middle face destruction with an association to Epstein-Barr virus. Fungi have been recovered from the diseased tissue now and then but were often seen as a lymphoma-associated secondary infection. However, there are ENKTL-NT cases with the recoveries of fungi and complete recovery with antifungal therapy, which are quite similar to rhino-orbital-cerebral mycosis (ROCM) that often confuses the physicians.MethodsWe searched Medline for English-language manuscripts limited to “human” and “case reports,” “letters,” “reviews,” and “clinical conferences” from 1966 to 2022. We used MeSH terms “lymphoma, extranodal nk-t-cell” [MeSH Terms] or “lethal midline granuloma” [MeSH Terms], in combination with MeSH terms “microbiology” [subheading] or “microbiology” [all fields] or “fungi” [all fields] or “fungi” [MeSH Terms] for ENKTL-NT with infections. We used MeSH terms “Mycoses” in combination with “Nose” [Mesh] OR “Orbital Diseases” [Mesh] for rhino-orbital-cerebral fungal infections.ResultsWe appraised 149 included articles and extracted references related to ENKTL-NT and/or ROCM. Themes and subcategories were subsequently derived. Our findings revealed that ROCM and ENKTL-NT are characterized by progressive and destructive ulcers in the midline face or rhino-orbital structures. ROCM is mainly caused by fungi in the order of Mucorales, and ENKTL-NT is usually associated with Epstein-Barr virus and sometimes fungi. Radiologically, both are characterized by non-specific features of sinusitis, soft tissue infection, and necrosis. Pathologically, ROCM and ENKTL-NT share the same characteristics of inflammation, necrosis, and granuloma. ROCM is characterized by the detection of fungi in tissue, while ENKTL-NT is typically positive for NK/T-cell markers and cytotoxic granule-associated proteins, proliferation, and vascular damage of angioinvasion, which could be incited by Mucor irregularis and Rhizopus arrhizus in patients and mice.ConclusionENKTL-NT and ROCM share many similarities in clinical presentations, radiology, and histopathology, and might have the same etiology. This may explain why the two diseases are tangled together in the reported cases, and suggests the role that the fungi may play in the development of these ENKTL-NT/ROCM diseases. The reason why ENKTL-NT and ROCM are sometimes confused is that the main pathogens of ROCM, Mucor irregularis and Rhizopus arrhizus, are the fungal causative agents of ENKTL-NT.

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Mucormycosis or extranodal natural killer/T cell lymphoma, similar symptoms but different diagnosis

Zhang

Wang

Liu

et al. 2016

Journal de Mycologie Médicale / Journal of Medical Mycology

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Nasal NK/T cell lymphoma with severe facial disfigurement in a 37-year-old male

Cited by 3 publications

References 16 publications

Extranodal NK/T-cell Lymphoma, Nasal Type Accompanied by PR3-ANCA-associated Glomerulonephritis

Extranodal NK/T-cell Lymphoma, Nasal Type Accompanied by PR3-ANCA-associated Glomerulonephritis

Rhino-Orbital-Cerebral Mycosis and Extranodal Natural Killer or/and T-Cell Lymphoma, Nasal Type

Mucormycosis or extranodal natural killer/T cell lymphoma, similar symptoms but different diagnosis

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