Natural Killer–like T-Cell Lymphoma of the Small Intestine With a Distinct Immunophenotype and Lack of Association With Gluten-Sensitive Enteropathy

2003 ◽  
Vol 127 (3) ◽  
pp. e142-e146 ◽  
Author(s):  
Constance M. Yuan ◽  
Steven Stein ◽  
John H. Glick ◽  
Mariusz A. Wasik
Keyword(s):  
Small Intestine ◽  
T Cell ◽  
Natural Killer ◽  
Chromosomal Abnormalities ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Chain Gene ◽  
Antigliadin Antibodies ◽  
Gluten Sensitive Enteropathy

Abstract We report the case of a large natural killer (NK)–like T-cell lymphoma that involved the ileum and displayed a distinct immunophenotype and complex karyotype. The patient exhibited no evidence of gluten-sensitive enteropathy (celiac disease) or any other type of enteropathy as determined by clinical history, endoscopy, and serology for immunoglobulin A (IgA) antiendomysial and IgG antigliadin antibodies. Molecular studies demonstrated a clonal T-cell receptor γ chain gene rearrangement. Immunophenotype analysis showed expression of intestinal epithelium-homing receptor CD103, CD7, cytoplasmic CD3ɛ, CD56, and CD16 but no other T- or NK-cell markers. Cytogenetic analysis of the malignant cells revealed multiple chromosomal abnormalities indicative of a biologically advanced, high-grade lymphoma. A novel subset of normal intestinal intraepithelial lymphocytes, bearing a similar phenotype, has been described; moreover, this subset diminishes, rather than expands, in gluten-sensitive enteropathy. This case supports the notion that lymphomas involving the small intestine represent a heterogeneous group of lymphomas with diverse pathogenetic mechanisms.

scholarly journals Nonnasal Lymphoma Expressing the Natural Killer Cell Marker CD56: A Clinicopathologic Study of 49 Cases of an Uncommon Aggressive Neoplasm

Blood ◽  
1997 ◽  
Vol 89 (12) ◽  
pp. 4501-4513 ◽  
Author(s):  
John K.C. Chan ◽  
V.C. Sin ◽  
K.F. Wong ◽  
C.S. Ng ◽  
William Y.W. Tsang ◽  
...  
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Cell Leukemia ◽  
Neoplastic Cells ◽  
Prolymphocytic Leukemia ◽  
Nasal Type ◽  
Nk T Cell

Abstract Expression of the natural killer (NK) cell antigen CD56 is uncommon among lymphomas, and those that do are almost exclusively of non–B-cell lineage and show a predilection for the nasal and nasopharyngeal region. This study analyzes 49 cases of nonnasal CD56+ lymphomas, the largest series to date, to characterize the clinicopathologic spectrum of these rare neoplasms. All patients were Chinese. Four categories could be delineated. (1) Nasal-type NK/T cell lymphoma (n = 34) patients were adults 21 to 76 years of age (median, 50 years), including 25 men and 9 women. They presented with extranodal disease, usually in multiple sites. The commonest sites of involvement were skin, upper aerodigestive tract, testis, soft tissue, gastrointestinal tract, and spleen. Only 7 cases (21%) apparently had stage I disease. The neoplastic cells were often pleomorphic, with irregular nuclei and granular chromatin, and angiocentric growth was common. The characteristic immunophenotype was CD2+ CD3/Leu4− CD3ε+ CD56+, and 32 cases (94%) harbored Epstein-Barr virus (EBV). Follow-up information was available in 29 cases: 24 died at a median of 3.5 months; 3 were alive with relapse at 5 months to 2.5 years; and 2 were alive and well at 3 and 5 years, respectively. (2) Aggressive NK cell leukemia/lymphoma (n = 5) patients presented with hepatomegaly and blood/marrow involvement, sometimes accompanied by splenomegaly or lymphadenopathy. The neoplastic cells often had round nuclei and azurophilic granules in the pale cytoplasm. All cases exhibited an immunophenotype of CD2+ CD3/Leu4− CD56+ CD16− CD57− and all were EBV+. All of these patients died within 6 weeks. (3) In blastoid NK cell lymphoma (n = 2), the lymphoma cells resembled those of lymphoblastic or myeloid leukemia. One case studied for CD2 was negative and both cases were EBV−. One patient was alive with disease at 10 months and one was a recent case. (4) Other specific lymphoma types with CD56 expression (n = 8) included one case each of hepatosplenic γδ T-cell lymphoma and S100 protein+ T-cell lymphoproliferative disease and two cases each of T-chronic lymphocytic/prolymphocytic leukemia, lymphoblastic lymphoma, and true histiocytic lymphoma. All of these cases were EBV−. Six patients died at a median of 6.5 months. Nonnasal CD56+ lymphomas are heterogeneous, but all pursue a highly aggressive clinical course. The nasal-type NK/T-cell lymphoma and aggressive NK cell leukemia/lymphoma show distinctive clinicopathologic features and a very strong association with EBV. Blastoid NK cell lymphoma appears to be a different entity and shows no association with EBV.

Natural Killer-Like T Cell Lymphoma of the Small Intestine: Report of a Case

Surgery Today ◽  
2006 ◽  
Vol 36 (5) ◽  
pp. 474-477 ◽  
Author(s):  
Yoshio Deguchi ◽  
Kazuhiko Yoshimatsu ◽  
Shungo Endo
Keyword(s):  
Small Intestine ◽  
T Cell ◽  
Natural Killer ◽  
Cell Lymphoma ◽  
T Cell Lymphoma

scholarly journals Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Harinder Gill ◽  
Raymond H. S. Liang ◽  
Eric Tse
Keyword(s):  
Stem Cell ◽  
T Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Natural Killer ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Hematopoietic Stem ◽  
Nasal Type

The World Health Organization (WHO) classification recognizes 2 main categories of natural killer (NK) cell-derived neoplasms, namely, extranodal NK/T-cell lymphoma, nasal type, and aggressive NK-cell leukaemia. Extranodal nasal NK/T-cell lymphoma is more frequent in the Far East and Latin America. Histopathological and immunophenotypical hallmarks include angiocentricity, angiodestruction, expression of cytoplasmic CD3 epsilon (ε), CD56, and cytotoxic molecules and evidence of Epstein-Barr virus (EBV) infection. Early stage disease, in particular for localized lesion in the nasal region, is treated with chemotherapy and involved-field radiotherapy. On the other hand, multiagent chemotherapy is the mainstay of treatment for advanced or disseminated disease. L-asparaginase-containing regimens have shown promise in treating this condition. The role of autologous hematopoietic stem cell transplantation is yet to be clearly defined. Allogeneic hematopoietic stem cell transplantation, with the putative graft-versus-lymphoma effect, offers a potentially curative option in patients with advanced disease.

scholarly journals MYC Functions As a Master Switch for Natural Killer Cell-Mediated Immune Surveillance of Lymphoid Malignancies

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2619-2619
Author(s):  
Srividya Swaminathan ◽  
Line Dam Heftdal ◽  
Daniel F Liefwalker ◽  
Renumathy Dhanasekaran ◽  
Anja Deutzmann ◽  
...  
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
Immune Surveillance ◽  
T Cell Lymphoma ◽  
Host Immune System ◽  
Type I ◽  
Lymphoid Malignancies ◽  
Cell Based Therapy

Abstract Background: The MYC oncogene drives T and B cell lymphoid malignancies, including Burkitt's lymphoma (BL) and Acute Lymphoblastic Leukemia (ALL). Such lymphomas are said to be "oncogene-addicted" to MYC. In order to develop targeted therapies for MYC-driven cancers, it is vital to understand how MYC regulates both cell autonomous and non-cell-autonomous processes, including host immunity. Approach: We have used a particularly tractable approach for studying the role of oncogenic MYC on the host immune system during lymphomagenesis through a tetracycline (tet)-system regulated transgenic mouse model of MYC-driven T cell lymphoma (SRα-tTA/Tet-O-MYC mice; Felsher and Bishop, Molecular Cell, 1999). By delineating the global immunological changes during primary MYC-driven T cell lymphomagenesis in SRα-tTA/Tet-O-MYC mice using mass cytometry (CyTOF) and CIBERSORT, we identified anti-tumor immune subsets that can be developed as therapies to treat MYC-driven lymphomas. Results: Amongst the immune subsets evaluated, our results demonstrated a specific systemic suppression of natural killer (NK) cell-mediated surveillance in SRα-tTA/Tet-O-MYC mice bearing overt MYC-driven T cell lymphomas. Inactivation of lymphoma-intrinsic MYC restores NK cell-mediated immune surveillance, suggesting that the regulation of NK cell-mediated surveillance may be integral to MYC-induced lymphomagenesis. We observed that lymphoma-specific MYC transcriptionally represses STAT1/2 and secretion of Type I Interferons (IFNs) required for normal NK cell homeostasis and NK cell-mediated immune surveillance in the tumor microenvironment. Concordantly, treating T cell lymphoma-bearing SRα-tTA/Tet-O-MYC mice with Type I IFN improves survival by rescuing NK cell production, and in part overriding MYC-mediated suppression of NK surveillance. We showed that human lymphomas with both high levels of MYC and low levels of STAT1/2 have lower NK surveillance, and are associated with poor prognosis. Finally, we established the therapeutic implications of our findings by showing that adoptive transfer of NK cells significantly delays lymphoma initiation, and recurrence after MYC inactivation; suggesting that NK cell-based therapy may be effective against MYC-driven lymphomas. Conclusion: Subversion of NK cell surveillance is integral to MYC-induced lymphomagenesis. Our studies provide a rationale for further developing the NK subset as a cell-based immunotherapy to effectively treat MYC-driven lymphomas in the future. Disclosures No relevant conflicts of interest to declare.

Nasal/Nasal Type NK/T-Cell Lymphoma: A Brazilian Experience of 40 Cases.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4627-4627
Author(s):  
Carlos Chiattone ◽  
Roberto P. Paes ◽  
Lidio Granato ◽  
Vania T.M. Hungria ◽  
Fausto F. Alonso ◽  
...  
Keyword(s):  
T Cell ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
Cell Lineage ◽  
T Cell Lymphoma ◽  
Chain Gene ◽  
Gamma Chain ◽  
Sphenoidal Sinus ◽  
Nasal Type ◽  
Nk T Cell

Abstract NK/T nasal type lymphoma is a rare type of non-Hodgkin’s lymphoma with a peculiar geographical distribution. Most of the reported cases come from Asian coutries and native populations from Latin America, such as Peru, Guatemala and Mexico. It is rarely reported in Brazilian population. We retrospectively studied 40 cases of Nasal/Nasal type NK/T-cell Lymphoma diagnosed at our center, between 1979 and 1999. Patient characteristics were as follows: 18 white,13 mulatto, 6 Asian and 3 black; 28 men and 12 women; median age 42.8 years (range 17–78). Patients originaly from all Brazilian geopolitical regions were present. One or more structures of the mild-facial region were involved in all cases, mainly in the nasal cavity (38 cases), ethmoidal sinus (21 cases), maxillary sinus (20 cases), palate (20 cases), nasopharingeal (13 cases), nose flap (11 cases), orbita (11 cases), sphenoidal sinus (9 cases), frontal sinus (6 cases), oropharingeal (3 cases). The most frequent symptoms were:nasal obstruction(27 cases), palate ulcer or edema(18 cases), fetid odor(14 cases), rhinorrehoea (9 cases), and orbital edema(8 cases). Considering the stage, 32 patients were in stage IE, 5 in stage IV, 1 in stage IIE and for 2, the stage was unknown. The median time from the onset of symptoms to diagnosis was 5.5 months, ranging between 1 and 17 months. The number of biopsies to confirm the diagnosis ranged between 1 and 5, with a median of 2 biopsies. Diagnosis was based on histological examination, immunohistochemistry panel(CD20, CD2, cCD3, CD43, CD45RO, CD16, CD56, granzin-B, TIA-1 and p53), in situ hybridization(ISH)forEBV and PCR for TCR gamma chain gene rearrangement. Important histological features were necrosis in almost all cases(38 cases) and angiocentricity and angioinvasion in 32 cases. There was no clear association between necrosis and angiocentricity, suggesting that other causes can be involved in the pathogenesis of the necrosis. Association between EBV and the pesence of necrosis in the histological study was present. Most of the cases showed a phenotypical and genotypical profile of NK cell lineage. Two cases presented monoclonal rearrangement for TCR gamma chain gene, characterizing T cell lineage. The only two cases of T cell lineage were also negative for EBV. Immunohistochemical reativity for p53 was observed in 24 cases. There was no association between the progression of the disease and the immunoexpression for p53. Most of the patients were treated with chemotherapy alone(14 cases) or associated with radiotherapy(20 cases). One patient was treated with radiotherapy alone and 5 patients were not treated due to a rapid progression to death. The overall median survival time was 12.6 months. The cause of death could be assessed in 23 patients;15 died due to infectious complications, and septicemia was the most frequent cause. The results of this retrospective survey confirmed that Nasal/Nasal type T/NK-cell Lymphoma is characterized by a very bad prognosis. More data are needed to know the epidemiology of this rare lymphoma in South America. The results show that EVB is also highly associated with this lymphoma in Brasilian patients.

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