Nasal T cell lymphoma (published in 2008)

2008-05-01   Francesco Cavazzini , Gian Matteo Rigolin , Antonio Cuneo 

1.Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, Ferrara Italy

Clinics and Pathology

Disease

Extranodal NH/T-cell lymphoma, nasal type.

Phenotype stem cell 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

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).

Bibliography

Pubmed IDLast YearTitleAuthors
93594781997Nasal T/natural killer (NK)-cell lymphomas are derived from Epstein-Barr virus-infected cytotoxic lymphocytes of both NK- and T-cell lineage.Chiang AK et al
113098172001Comparative genomic hybridization study of nasal-type NK/T-cell lymphoma.Ko YH et al
160499162005Genome-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 et al
104145051999Histological and immunophenotypic profile of nasal NK/T cell lymphomas from Peru: high prevalence of p53 overexpression.Quintanilla-Martinez L et al
117863992002Specific patterns of gene methylation in natural killer cell lymphomas : p73 is consistently involved.Siu LL et al
105502951999Comparative genomic hybridization analysis of natural killer cell lymphoma/leukemia. Recognition of consistent patterns of genetic alterations.Siu LL et al
93261901997Identification of del(6)(q21q25) as a recurring chromosomal abnormality in putative NK cell lymphoma/leukaemia.Wong KF et al
104393611999Cytogenetic abnormalities in natural killer cell lymphoma/leukaemia--is there a consistent pattern?Wong KF et al
102332751999Nasal-type T/natural killer cell angiocentric lymphoma, Epstein-Barr virus-associated, and showing clonal T-cell receptor gamma gene rearrangement.Yoon TY et al

Summary

Note

see the more recent paper on Extranodal NK/T-cell lymphoma

Citation

Francesco Cavazzini ; Gian Matteo Rigolin ; Antonio Cuneo

Nasal T cell lymphoma (published in 2008)

Atlas Genet Cytogenet Oncol Haematol. 2008-05-01

Online version: http://atlasgeneticsoncology.org/haematological/2100/nasal-t-cell-lymphoma-(published-in-2008)

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