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April
2007
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Gastrointestinal tract is the most common
site of primary extranodal lymphoma . Lymphoma accounts for 30% of small
bowel malignancies. Primary gastrointestinal lymphoma exhibits no
evidence of liver, spleen or bone marrow involvement at the time of
diagnosis. Regional lymphnode involvement may be present.
Criteria used to classify malignant lymphoma of small bowel include:
i) Cell lineage: B or T cell lymphoma
ii) Primary or systemic nature of tumour
iii)Associated backround chronic illnesses.
Primary gastrointestinal lymphoma usually arise as a sporadic tumour.
Sporadic lymphomas, also termed the 'Westerm type' are B- cell
lymphomas which appear to arise from the B- cells of the mucosa
associated lymphoid tissue.
Some lymphomas develop in the backround of chronic diseases . Eg.
Sprue -like malabsorption syndromes, congenital immunodeficiency states,
infection with HIV following organ transplantation with immunosuppresion
(heart transplant recipients), those complicating Crohn's disease ,
hypogammaglobulinaemia with lymphoid hyperplasia, in patients with alpha
chain disease.
Ileum is commonly affected followed by jejunum and duodenum. |
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Immunoproliferative small
intestinal disease, / Mediterranean lymphoma:
B-cell lymphoma usually arises in
children and young adults with Mediterranean ancestry.
Usually located in the distal portion of duodenum and upper jejunum.
Diffusely thickened bowel wall with small nodules or discrete tumour
masses indicate high grade tumour.
Diagnosis can be made by endoscopic biopsy. Histologically the low
grade tumour is characterized by an infiltrate of plasma cells and
lympho-plasmacytoid cells in the mucosa. There is broadening &
shortening of villi and separation of crypts. The cells appear mature. In
the early stage the tumour responds to antibiotics.
High grade tumour is characterized by pleomorphic large cells with
immunoblastic and plasmacellular features.
Immunohistochemistry
reveals alpha heavy chains of immunoglobulins in
the cytoplasm. |
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Burkitt's lymphoma
of small bowel:
-Relatively common in the Middle east and Algeria.
-Usually located in the ileum and the ileocaecal region.
-May cause intussusception.
-Histologically, the tumour consists of lymphoblast like cells with high
mitotic rate
interspersed with macrophage containing cellular debris (starry-sky).
The infiltrating tumour dissects through the muscle fibres and
surrounds the regional lymphnodes without infiltrating them.
-Immunohistochemistry: CD19,20,22 &79a : positive
CD 10: positive,
CD5 & 23: negative
Ki67 : more than 85% cell are positive. |
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Mantle cell
lymphoma of small bowel:
Presents as lymphoid polyps in elderly patients (around 60
years of age).
It is usually associated with leukemic spread.
Immunohistochemistry:
Cyclin D1 positive
CD5 positive
CD10 : negative / positive
CD19,20,22, 79a positive
CD23 : negative |
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Follicular lymphoma
of the small bowel:
The tumour has a follicular growth pattern and consists
of a mixture of germinal
centre blasts and cleaved
cells ( centroblasts and centrocytes).
Immunohistochemistry:
CD5 : negative
CD10 : positive/negative
CD19, 20, 22, 79a : positive
BCL-2 : positive
Occasionally, these tumours transform into diffuse tumour containing
large centroblasts.
(Diffuse large B- cell lymphoma). |
- Lymphoid
hyperplasia of small intestine is a reactive process.
Path Quiz Case51
- Usually located at the ileocecal junction.
- Clinically the patients present with right iliac fossa pain,
obstruction and sometimes intussusception.
- Associated with viral infection in children.
- Diffuse nodular lymphoid hyperplasia is present in some patients of
primary hypogammaglobulinemia.
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Giardia lamblia infection is noted in some patients with lymphoid
hyperplasia.
- D/D: Malignant lymphoma: Immunohistochemistry is performed to confirm
the diagnosis |
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IMAGES
Immunoproliferative small intestinal disease



Mantle cell lymphoma

Follicular lymphoma of
terminal ileum

Burkitt's lymphoma
of small intestine

Lymphoid hyperplasia of small bowel
(pseudolymphoma)

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