Primary gastrointestinal lymphomas
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Інститут експериментальної патології, онкології і радіобіології ім. Р.Є. Кавецького НАН України
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| Zitieren: | Primary gastrointestinal lymphomas / E. Vandenberghe // Experimental Oncology. — 2012. — Т. 34, № 4. — С. 385-386. — Бібліогр.: 9 назв. — англ. |
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irk-123456789-1398572018-06-22T03:05:15Z Primary gastrointestinal lymphomas Vandenberghe, E. Conference reports 2012 Article Primary gastrointestinal lymphomas / E. Vandenberghe // Experimental Oncology. — 2012. — Т. 34, № 4. — С. 385-386. — Бібліогр.: 9 назв. — англ. 1812-9269 http://dspace.nbuv.gov.ua/handle/123456789/139857 en Experimental Oncology Інститут експериментальної патології, онкології і радіобіології ім. Р.Є. Кавецького НАН України |
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Conference reports Conference reports |
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Conference reports Conference reports Vandenberghe, E. Primary gastrointestinal lymphomas Experimental Oncology |
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Article |
| author |
Vandenberghe, E. |
| author_facet |
Vandenberghe, E. |
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Vandenberghe, E. |
| title |
Primary gastrointestinal lymphomas |
| title_short |
Primary gastrointestinal lymphomas |
| title_full |
Primary gastrointestinal lymphomas |
| title_fullStr |
Primary gastrointestinal lymphomas |
| title_full_unstemmed |
Primary gastrointestinal lymphomas |
| title_sort |
primary gastrointestinal lymphomas |
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Інститут експериментальної патології, онкології і радіобіології ім. Р.Є. Кавецького НАН України |
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2012 |
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Conference reports |
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http://dspace.nbuv.gov.ua/handle/123456789/139857 |
| citation_txt |
Primary gastrointestinal lymphomas / E. Vandenberghe // Experimental Oncology. — 2012. — Т. 34, № 4. — С. 385-386. — Бібліогр.: 9 назв. — англ. |
| series |
Experimental Oncology |
| work_keys_str_mv |
AT vandenberghee primarygastrointestinallymphomas |
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2025-07-10T09:14:37Z |
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2025-07-10T09:14:37Z |
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1837250782487904256 |
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Experimental Oncology ��� �������� ���� ��ecem�er���� �������� ���� ��ecem�er� ��ecem�er� ��5
lymphocytes are usually small or morphologically “vil-
lous”� and the leukemic manifestation of SMZL is named
splenic lymphoma with villous lymphocytes �SLVL�. The
typical immunophenotype is C��9+C���+C���+C��5+
and the clone is often also C� ���+ and C���+. More-
over� C���c is highly associated with SMZL. The genet-
ics and pathogenesis of SMZL are poorly understood
and specific prognostic features are lacking. A�errant
karyotypes are seen as gains of �/�q and ��q� deletions
of �q and 6q and translocations involving �q/�q/l�q.
Trisomy � and deletions of chromosome �q��-�� are
most common and found in approximately �5 and �5%
of cases� respectively. A strong association has �een
descri�ed �etween usage of the IGVH�-� and deletion
�q and ��q alterations. Clinical and epidemiological
data suggest that chronic hepatitis C virus �HCV� infec-
tion may have an etiological role in a su�set of cases.
MicroRNA �miR�-�6�� a miRNA known to have tumor
suppressive properties� has �een shown to �e down-
regulated in HCV positive cases. Recent data suggest
that certain SMZL su�types could derive from progeni-
tor populations adapted to particular antigenic chal-
lenges through selection of VH domain specificities�
in particular the IGHV �-��*��� allele.
The anti-C��� anti�ody rituxima� is mostly ef-
fective in MZL patients as monotherapy� �ut for many
patients with symptomatic splenomegaly� splenectomy
is still a therapeutic option.
In summary� the presence of lymphocytosis in the
�lood in patients with a suspicion of lymphoma requires
careful evaluation for the presence of neoplastic lym-
phocytes� especially in the a�sence of easily acces-
si�le enlarged lymph nodes. The differential diagnosis
�etween the WHO defined mature B-cell malignancies
has improved �y using multiple-color flow cytometry
of phenotypic data of the lymphoma cells. This method
is also of value for characterization of the immune
cells in the microenvironment and �lood. Molecular/
cytogenetic analyses have a role in classification
of the disease and for understanding of pathogenesis.
Therapeutic decisions are always dependant on the
specific diagnosis� prognostic factors and a careful
clinical evaluation of the patient.
REFERENCES
1. Bene MC, Nebe T, Bettelheim P, et al. Immunophe-Immunophe-
notyping of acute leukemia and lymphoproliferative disor-
ders: a consensus proposal of the European LeukemiaNet
Work Package 10. Leukemia 2011; 25: 567–74.
2. Wahlin BE, Sundstrom C, Holte H, et al. T cells
in tumors and blood predict outcome in follicular lymphoma
treated with rituximab. Clin Cancer Res. 2011; 17: 4136–44
3. Matutes E, Parry-Jones N, Brito-Babapulle V, et al.
The leukemic presentation of mantle-cell lymphoma: disease
features and prognostic factors in 58 patients. Leuk Lymphoma
2004; 45: 2007–15.
4. Fernandez V, Salamero O, Espinet B, et al. Genomic
and gene expression profiling defines indolent forms of mantle
cell lymphoma. Cancer Res. 2010; 70: 1408–18.
5. Nygren L, Baumgartner Wennerholm S, et al. Prognostic
role of SOX I I in a population-based cohort of mantle cell
lymphoma. Blood 2012; 119: 4215–23.
6. Del Giudice I, Messina M, Chiaretti S, et al. Behind the
scenes of non-nodal MCL: downmodulation of genes involved
in actin cytoskeleton organization, cell projection, cell adhesion,
tumour invasion, TP53 pathway and mutated status of immu-
noglobulin heavy chain genes. J Haematol 2012; 156: 601–11.
7. Isaacson PG, Matutes E, Burke M, Catovsky D. The
histopathology of splenic lymphoma with villous lymphocytes.
Blood 1994; 84: 3828–34.
8. Matutes E, Morilla R, Owusu-Ankomah K, et al. The
immunophenotype of splenic lymphoma with villous lympho-
cytes and its relevance to the differential diagnosis with other
B-cell disorders. Blood 1994; 83: 1558–62.
9. Catovsky D, Matutes E. Splenic lymphoma with circu-
lating villous lymphocytes/splenic marginal zone lymphoma.
Seminars in Hematology 1999; 36: 148–54.
10. Chacon JI, Mollejo M, Munoz E, et al. Splenic mar-Splenic mar-
ginal zone lymphoma: clinical characteristics and prognostic
factors in a series of 60 patients. Blood 2002; 100: 1648–54.
11. Parry-Jones N, Matutes E, Gruszka-Westwood AM, et al.
Prognostic features of splenic lymphoma with villous lympho-
cytes: a report on 129 patients. Brit J Haematol 2003; 120: 759–64.
12. Del Giudice I, Matutes E, Morilla R, et al. The diag-The diag-
nostic value of CD 123 in B-cell disorders with hairy or villous
lymphocytes. Haematologica 2004; 89: 303–8.
13. Matutes E, Oscier D, Montalban C, et al. Splenic
marginal zone lymphoma proposals for a revision of diagnostic,
staging and therapeutic criteria. Leukemia 2008; 22: 487–95.
14. Salido M, Baro C, Oscier D, et al. Cytogenetic aber-Cytogenetic aber-
rations and their prognostic value in a series of 330 splenic
marginal zone B-cell lymphomas: a multicenter study of the
Splenic B-Cell Lymphoma Group. Blood 2010; 116: 1479–88.
PRIMARY GASTROINTESTINAL
LYMPHOMAS
E. Vandenberghe
Haematology/Oncology Day Care Centre, St.
James’s Hospital, Dublin, Ireland
Correspondence: vandenberghesec@stjames.ie
Epidemiology and classification
Primary gastrointestinal lymphomas comprise less
than 5% of all lymphomas diagnosed in the western
world� with a varia�le geographical and ethnic inci-
dence. The �iology and management vary with the
main diagnostic �WHO� su�types which include Gastric
MALT lymphomas� Enteropathy associated T-cell lym-
phoma �EATCL�. Other lymphomas which frequently
involve the gastrointestinal tract include diffuse large
B-cell lymphoma� mantle cell lymphoma and Burkitts
lymphoma; �ut are not considered primary gut lym-
phomas and will not �e covered in this lecture. The
pathogenesis of MALT and EATCL lymphomas is linked
to a�normal antigen drive �gluten/Helicobacter infec-
tion� resulting in chronic inflammation and lymphoma
development. The lymphomas are otherwise radically
different; MALT lymphomas are indolent B-NHL� which
respond to antigen-drive withdrawal and minimal
therapy with an overall survival �OS� > ��% at 5 years�
whereas EATCL is an aggressive T-cell lymphomas
associated with a poor outcome.
Gastric MALT lymphoma
Clinical features: Gastric MALT lymphomas in-
cidence in the Western World is approximately 6 per
��6 Experimental Oncology ��� �������� ���� ��ecem�er�
million� with a median onset at 6� years� slight female
predominance and almost invaria�le association with
Helico�acter pylori infection. Patients typically present
with non-specific dyspeptic type symptoms and the
diagnosis is made gastroscopically. ��% of patients
have Stage I/II disease.
Pathology: The pathological appearance
is of small- to medium-sized round or minimally irregu-
lar cells� with clumped nuclear chromatin� a�undant
pale cytoplasm and lymphoepithelial lesions. The cells
express pan-B markers �ut are C�5� �� and �� nega-
tive. The t���;���q��;q��� detecta�le �y FISH is pre-
sent in up to 5�% of cases with PCR-detecta�le im-
munoglo�ulin gene rearrangements in 9�% of cases.
Management: H pylori eradication is standard
treatment for all patients and in those with disease
confined to the mucosa and su�mucosa results in a du-
ra�le CR in ��% of cases. For persistant or progressive
disease chemotherapy with Chloram�ucil +/- Ritux-
ima� or loco-regional radiotherapy with �� Gy are
standard approaches. There is no evidence that more
intensive therapy results in a �etter outcome. Life long
follow-up should include regular endoscopy.
Enteropathy-associated T cell lymphoma
Clinical features: Coeliac disease �C�� is caused
�y gluten intolerance resulting in small intestinal su�-
villous atrophy and mala�sorption of varia�le severity
which is managed with a gluten free diet �GF��. Coeliacs
have a �� fold increased rate of developing lymphoma
with 6���5% of them su�-typed as EATCL. Clinical pre-
sentation follows � patterns ��� development of refrac-
tory coeliac disease �RC�II� despite adherence to a GF�
��� acute presentation with gut perforation/acute severe
mala�sorption despite adherence to a GF� and ���
acute presentation as in ��� with no previous diagnosis
of C�. EATCL diagnosis can �e challenging as it is usu-
ally confined to the small intestine and tissue is usually
o�tained surgically or �y endoscopy �gastroscopy/
dou�le �alloon entersocopy�.
Pathology: EATCL is characterised �y a monomor-
phic population of medium to large cells with round
or angulated vesicular nuclei� prominent nucleoli and
moderate to a�undant� pale-staining cytoplasm with ex-
pression of C��+� C�5+� C��+� C��+/-� C��- and C����+.
Management: The 5 year OS is ��% with conven-
tional chemotherapy and this poor outcome is thought
to �e related to poor patient performance status
secondary to nutritional deficiency/gastrointestinal
surgery and the chemo-refractoriness inherent to T-cell
lymphomas. Outcome can �e improved using intensive
nutritional support and primary chemotherapy followed
�y an autologous transplantation for patients under the
age of 65 resulting in a 5 year OS of �etween 5��6�%.
Refractory coeliac disease: Patients who are
diagnosed with an RC�II prodrome are interesting
�oth for insights into EATCL lymphomagenesis and
also �ecause they may respond to less intensive
therapy� thus reducing the risk of EATCL transforma-
tion. RC� II is characterised �y su�-villous atrophy�
loss of C�� intra-epithelial lymphocytes and clonal
T-lymphocytes with ��% progression to EATCL within
5 years. A small study of patients with RC�II who re-
sponded to Cladri�ine therapy had a 5 year OS of ��%
which may �e improved further �y autologous SCT.
REFERENCES
1. Falk S. Lymphomas of the upper GI tract: the role
of radiotherapy. Clin Oncol (R Coll Radiol) 2012; 30: 1–6.
2. Zucca E, Dreyling M on behalf of the ESMO Guidelines
Working Group. Ann Oncology 2010; 21: 175–6.
3. Martinell I, Laszlo D, Ferreri AJ, et al. Clinical acti-
vity of rituximab in gastric marginal zone lymphoma resistant
to or not eligible for anti-Helicobacter pylori therapy. J Clin
Oncol 2005; 23: 1979–83.
4. Levy M, Copie-Bergman C, Moliner-Frenkel V, et al.
Treatment of t(11;18) positive gastric mucosa associated lym-
phoid tissue lymphoma with rituximab and chlorambucil: clini-
cal, histological and molecular follow up. Leuk Lymphoma
2010; 51: 284–90.
5. Morgner A, Schmelz R, Thiede C, et al. Therapy of gas-Therapy of gas-
tric mucosa associated lymphoid tissue lymphoma. World
J Gastroenterol 2007; 13: 3554–66.
6. Sabatino A, Biagi F, Gobbi P, et al. How I treat enter-How I treat enter-
opathy associated T cell lymphoma. Blood 2011; 119: 2458–67.
7. Rubio-Tapia A, Murray J. Classification and manage-
ment of refractory celiac disease. GUT 2010; 59: 547–57.
8. Bishton M, Haynes A. Combination chemotherapy
followed by autologous stem cell transplant for enteropathy
associated T cell lymphoma. Br J Haem 2006; 136: 111–3.
9. Tack G, Verbeek W Al-Toma A, et al. Evaluation
of Cladribine treatment in refractory celiac disease type
II. World J Gastroenterol 2011; 17: 506–13.
MATURE T- AND NK- CELL NEOPLASMS
C. Dearden
Royal Marsden Hospital NHS Foundation Trust,
London, United Kingdom
Correspondence: claire.dearden@rmh.nhs.uk
The mature or peripheral T-cell neoplasms are
a �iologically and clinically heterogeneous group of rare
disorders that result from clonal proliferation of mature
post-thymic lymphocytes. Natural killer �NK� cells are
closely related to T cells and neoplasms derived from
these are therefore considered within the same group.
The World Health Organization �WHO� classification
of haemopoietic malignancies has divided this group
of disorders into those with predominantly leukaemic
�disseminated�� nodal� extra-nodal or cutaneous presen-
tation. Within the WHO classification these malignancies
are differentiated on the �asis not only of clinical features
�ut also of morphology� immunophenotype and genetics.
The mature T-cell and NK-cell neoplasms account
for approximately �����% of all lymphoid malignan-
cies� usually affect adults and most of the entities
descri�ed are more commonly reported in males than
in females. The median age at diagnosis for the group
as a whole is 6� years with a range of ���9� years.
There is geographical variation in the frequency of the
different su�types and in Europe peripheral T-cell
lymphoma� not otherwise specified �PTCL-NOS��
anaplastic large cell lymphoma �ALCL� and angio-
immuno�lastic T-cell lymphoma �AITL� account for
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