LXXXVII Lethal Granulomatous Ulceration Involving the Midline Facial Tissues

Williams, Henry L.

doi:10.1177/000348944905800405

Cited by 56 publications

(15 citation statements)

References 20 publications

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“…Until the late 20 th century, a fatal destructive tumor that develops around the nasopharyngeal and nasal/paranasal areas, sometimes penetrating the palate or the cranial dura mater, resulting in death was called lethal midline granuloma [1]. Study of cell surface markers in immunity has shown that expression of CD56, a marker of natural killer cells (NK cells) and some γδ T-cells, can be used to define this disease as extranodal natural killer/T-cell lymphoma, nasal type (NKTL) [2].…”

Section: Introductionmentioning

confidence: 99%

Novel Targets for Natural Killer/T-cell Lymphoma Immunotherapy

2015

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SummaryExtranodal natural killer/T-cell lymphoma, nasal type (NKTL) is a rare but highly aggressive Epstein-Barr virus-related malignancy, which mainly occurs in nasopharygeal and nasal/paranasal areas. In addition to its high prevalence in Asian, Central American and South American populations, its incidence rate has been gradually increasing in Western countries. The current mainstay of treatment is a combination of multiple chemotherapies and irradiation. Although chemoradiotherapy can cure NKTL, it often causes severe and fatal adverse events. Because a growing body of evidence suggests that immunotherapy is effective against hematological malignancies, this treatment could provide an alternative to chemoradiotherapy for treatment of NKTL. In this review, we focus on how recent findings could be used to develop efficient immunotherapies against NKTL.

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Section: Introductionmentioning

confidence: 99%

Novel Targets for Natural Killer/T-cell Lymphoma Immunotherapy

2015

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“…18 Because, macroscopically, the lesions usually looked like necrotic granuloma and the clinical course was shown to be aggressive and lethal, the clinical terms "rhinitis gangrenosa progressiva" 19 and "lethal midline granuloma", 20 were initially used for this condition in Europe and the United States, respectively. The histological features of the disease are characterized by angiocentric and polymorphous lymphoreticular infi ltrates with ischemic necrosis, resulting in diagnostic diffi culty, as exemplifi ed by the plethora of terms used for these lesions.…”

Section: Historical Backgroundmentioning

confidence: 99%

Nasal natural killer (NK)/T-cell lymphoma: clinical, histological, virological, and genetic features

et al. 2009

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Nasal natural killer (NK)/T-cell lymphoma (NNKTL) is a clinical illness characterized by progressive unrelenting ulceration and necrosis of the nasal cavity and midline facial tissues. Histological features of the lymphoma include angiocentric and polymorphous lymphoreticular infiltrates, called polymorphic reticulosis. Surface antigens and the NK-cell marker, CD56, as well as pan-T antigen CD2, cytoplasmic CD3 (CD3epsilon), and CD45 are expressed in the lymphoma cells. The origin of the lymphoma is thought to be either NK-cell linkage without T-cell receptor (TCR) rearrangement or gammadeltaT-cell linkage with gammadeltaTCR rearrangement. Since the authors of this study first demonstrated the presence of Epstein Barr virus (EBV)-DNA and EBV oncogenic proteins in NNKTL, the lymphoma has been classified as one of the EBV-associated malignancies. The NNKTL cells produce interleukin (IL)-9, IL-10, and interferon-gamma-inducible protein-10 (IP-10), possibly due to EBV-oncogenic proteins in the lymphoma cells, and such cytokines take an important part in the cell proliferation and invasion, acting in an autocrine manner. Clinically, the serum EBV-DNA copy number is useful as a specific tumor marker and a predictive prognostic factor. Even in early clinical stages, the lymphoma shows poor prognosis caused by the rapid progression of the lesion into distinct organs. Our newly designed arterial infusion chemotherapy, from the superficial temporal artery, in combination with radiotherapy, has shown a favorable outcome in patients with NNKTL. In this article, the clinical, pathological, and virological characteristics of the lymphoma are reviewed, along with a report of our investigations.

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“…Williams 7 stresses the possibility that it might be the result of hyperimmunity. He believes that the tissues in this area of the nose have an innate capacity to develop hyperimmunity, possibly greater than any other area of the body.…”

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confidence: 99%