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Nasal T cell lymphoma (published in 2008)

Written2008-05Antonio Cuneo, Francesco Cavazzini, Gian Matteo Rigolin
Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, Ferrara Italy

(Note : for Links provided by Atlas : click)


ICD-Morpho 9719/3 Extranodal NK/T-cell lymphoma, nasal type
Atlas_Id 2100
Note see the more recent paper on Extranodal NK/T-cell lymphoma
Other namesAngiocentric T-cell lymphoma
Polymorphic reticulosis
Lethal midline granuloma

Clinics and Pathology

Disease Extranodal NH/T-cell lymphoma, nasal type.
Phenotype / cell stem origin This lymphoma derives form the transformation of NK lymphocytes and, less frequently, T-lymphocytes.
Epidemiology It is seen most frequently in China, Japan, Korea and other Asian countries and in Central America.
Clinics Middle aged adults are most frequently affected, with slight male predominance. The disease involves the nasal cavity and may spread to the pharynx, palate and larinx. Less frequently, orbital and cranial nerve involvement was described. Spreading to the skin, soft tissue and gastrointestinal tract may occur. Bone marrow involvement is unusual at presentation. Hemophagocytic syndrome was described in some cases. The tumor is locally invasive and destructive (Liang et al., 2006).
Pathology The cellular infiltrate is polymorphic, typically associated with an angioinvase growth pattern, with consequent angiodestruction, ischemia and tissue necrosis. Neoplastic cells are CD56 positive, with negativity for surface CD3. The TCR gene is usually germline, even though some cases with a clonally rearranged TCR were reported (Yoon et al., 1999).
Epstein-Barr virus infection in this lymphoma was well documented by molecular methods (Chiang et al., 1997).
Treatment Combination regimens such as CHOP or other aggressive schedules followed by local radiotherapy are the mainstay of treatment. Autologous bone marrow transplantation has been used.
Prognosis Prognosis is severe, with less than 50% of the patients achieving durable complete response after intensive chemotherapy and local radiotherapy. The disseminated forms of the disease are almost uniformly fatal.


Cytogenetics Morphological Three out of seven cases studied by Wong et al (1997), including one nasal, one extranasal and one leukaemic form, showed a common region of deletion at 6q21-q25, suggesting that this may be a nonrandom chromosomal aberration.
Other non-random abnormalities include +X, i(1q), i(7q), +8, del(13q), del(17p), i(17q), and 11q23 rearrangement (Wong et al., 1999).
Cytogenetics Molecular P73 gene methylation was described in 94% of the cases; other methylated genes included hMLH1 (63%), p16 (63%), p15 (48%), and RAR beta (47%) (Siu et al., 2002). P53 gene overexpression was documented (Quintanilla-Martinez et al., 1999).
Comparative genomic hybridization studies identified del(6q), del(13q),del (17p), del (1p), del(12q), and partial gain of Xp, 2p, 10q as recurrent abnormalities (Siu et al., 1999; Ko et al., 2001). Some of these abnormalities (i.e. 17p deletion and 1p deletion) may be associated the aggressive leukemic variant of the disease (Nakashima et al., 2005).
Genome-wide array-based comparative genomic hybridization identified recurrent regions of imbalances: gain of 2q and loss of 6q16-27,11q22-23, 5p14, 5q34, 1p36, 2p16, 4q12, 4q31 (Nakashima et al., 2005).


Nasal T/natural killer (NK)-cell lymphomas are derived from Epstein-Barr virus-infected cytotoxic lymphocytes of both NK- and T-cell lineage.
Chiang AK, Chan AC, Srivastava G, Ho FC.
Int J Cancer 1997;73:332-338.
PMID 9359478
Comparative genomic hybridization study of nasal-type NK/T-cell lymphoma.
Ko YH, Choi KE, Han JH, Kim JM, Ree HJ.
Cytometry. 2001;46:85-91.
PMID 11309817
Nasal T7NK-cell lymphoma.
Liang RL.
In: Canellos GP, Lister TA, Young BD: The Lymphomas 2nd edition. Sanuders Elsevier, Philadelphia, 2006, pp 451-455
Genome-wide array-based comparative genomic hybridization of natural killer cell lymphoma/leukemia: different genomic alteration patterns of aggressive NK-cell leukemia and extranodal Nk/T-cell lymphoma, nasal type.
Nakashima Y, Tagawa H, Suzuki R, Karnan S, Karube K, Ohshima K, Muta K, Nawata H, Morishima Y, Nakamura S, Seto M.
Genes Chromosomes Cancer. 2005;44:247-255.
PMID 16049916
Histological and immunophenotypic profile of nasal NK/T cell lymphomas from Peru: high prevalence of p53 overexpression.
Quintanilla-Martinez L, Franklin JL, Guerrero I, Krenacs L, Naresh KN, Rama-Rao C, Bhatia K, Raffeld M, Magrath IT.
Hum Pathol. 1999;30:849-55.
PMID 10414505
Specific patterns of gene methylation in natural killer cell lymphomas : p73 is consistently involved.
Siu LL, Chan JK, Wong KF, Kwong YL.
Am J Pathol. 2002;160:59-66.
PMID 11786399
Comparative genomic hybridization analysis of natural killer cell lymphoma/leukemia. Recognition of consistent patterns of genetic alterations.
Siu LL, Wong KF, Chan JK, Kwong YL.
Am J Pathol. 1999;155:1419-25.
PMID 10550295
Identification of del(6)(q21q25) as a recurring chromosomal abnormality in putative NK cell lymphoma/leukaemia.
Wong KF, Chan JK, Kwong YL.
Br J Haematol. 1997;98:922-926.
PMID 9326190
Cytogenetic abnormalities in natural killer cell lymphoma/leukaemia--is there a consistent pattern?
Wong KF, Zhang YM, Chan JK.
Leuk Lymphoma. 1999;34:241-250.
PMID 10439361
Nasal-type T/natural killer cell angiocentric lymphoma, Epstein-Barr virus-associated, and showing clonal T-cell receptor gamma gene rearrangement.
Yoon TY, Lee HT, Chang SH.
Br J Dermatol. 1999; 140:505-508.
PMID 10233275


This paper should be referenced as such :
Cuneo, A ; Cavazzini, F ; Rigolin, GM
Nasal T cell lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2009;13(5):374-375.
Free journal version : [ pdf ]   [ DOI ]
On line version :

Other genes implicated (Data extracted from papers in the Atlas) [ 4 ]


External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMap (UZH-SIB Zurich)Topo ( C42) Morph ( 9719/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
REVIEW articlesautomatic search in PubMed
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