Nasal T/NK-Cell Lymphoma

Liang, Raymond

doi:10.1016/b978-0-7216-0081-9.50031-5

Cited by 2 publications

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“…Extranodal NK/T‐cell lymphoma, nasal type usually presents in the upper aero‐digestive tract, especially the nasal cavity. Historically, different names were used to describe this extraordinary tumour (Liang et al , 1987, 1990, 1995;Liang, 2000, 2006). It has been called lethal midline granuloma, midline malignant reticulosis and polymorphic reticulosis (Ho et al , 1990a; Chim et al , 1999a).…”

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Advances in the management and monitoring of extranodal NK/T‐cell lymphoma, nasal type

Liang

2009

Br J Haematol

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Summary Extranodal natural killer (NK)/T‐cell lymphoma, nasal type, has a unique geographic distribution. Its pathology is characterized by marked angio‐invasion and tissue necrosis. A typical NK‐cell phenotype is usually present: CD2+, CD3 epsilon+, CD56+, cytotoxic molecules+ and Epstein–Barr virus (EBV)+. Magnetic Resonance Imaging helps to clearly define the local involvement. Positron Emission Tomography helps to demonstrate system spread. Various prognostic variables (International Prognostic Index or the Korean Prognostic Index) should be documented. This may include quantification of plasma EBV DNA. For localized nasal disease, radiotherapy is important, although chemotherapy is often added. Sustainable remission is observed in over half of these patients. For extra‐nasal or disseminated disease, systemic chemotherapy becomes the mainstay and the prognosis is usually poor. Doxorubicin‐containing regimens are not entirely satisfactory and l‐asparaginase containing regimens are being investigated. Patients with poor prognostic features may be considered for an early autologous haematopoietic stem cell transplant. Allogenetic transplantation is efficacious but is associated with high transplant‐related mortality.

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Advances in the management and monitoring of extranodal NK/T‐cell lymphoma, nasal type

Liang

2009

Br J Haematol

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“…Clinically, the previously known as nasal NK/T-cell lymphoma arises frequently in the nasal cavity, nasopharynx, or upper aerodigestive tract, developing ulcerative and destructive lesions, spreading to the adjacent anatomic structures typically associating bone erosion. 18 Unlike this characteristic clinical behavior, our patient had a long period of 4 years with recurrent but nondestructive lesions that appeared at a very early age. When the nasal cavity was explored, no lesions were found.…”

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Hydroa Vacciniforme Lymphoproliferative Disorder in a Young Spanish Woman: An Infrequent Case With Fatal Outcome

Garcia-Garcia,

Morales Moya,

Val

et al. 2024

The American Journal of Dermatopathology

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Hydroa vacciniforme (HV) lymphoproliferative disorder is a rare NK/T-cell lymphoma mainly affecting children and with a clinical resemblance to HV, which is mostly reported in Latin American and some Asian countries. Overall, the mature T cell and NK-cell neoplasms are now grouped into 9 families based on diverse concepts: cell of origin/differentiation state, clinical scenario, disease localization, and cytomorphology. HV lymphoproliferative disorder is listed within the group of Ebstein Barr Virus-positive T-cell and NK-cell lymphoid proliferations and lymphomas of childhood according to the fifth edition of the World Health Organization Classification of mature lymphoid neoplasms. We report the extraordinary case of a 22-year-old white woman, native of Spain, first presented in 2016 when she started suffering from recurrent facial edema. Four years later, the disease progressed with lymph node spreading and a fatal outcome. Here, we describe the clinical and histological presentation of the lymphoma throughout its evolution. Cases like this can be difficult to classify posing a real challenge to clinicians and pathologists. So, it is vital to be aware of the rare presentation of this disease to be able to identify the clinical and histological picture to make a correct diagnosis and establish an early treatment.

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Nasal T/NK-Cell Lymphoma

Cited by 2 publications

References 58 publications

Advances in the management and monitoring of extranodal NK/T‐cell lymphoma, nasal type

Advances in the management and monitoring of extranodal NK/T‐cell lymphoma, nasal type

Hydroa Vacciniforme Lymphoproliferative Disorder in a Young Spanish Woman: An Infrequent Case With Fatal Outcome

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