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Pathology of Intestinal Lymphoma

 

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B-Cell Lymphoma :

- Maltoma; Gastric Lymphoma

- Immunoproliferative Small Intestinal Disease

- Burkitt's Lymphoma

- Mantle Cell Lymphoma

- Follicular Lymphoma

- Plasmacytoma

- Other B-Cell Lymphomas (mostly high grade)

T- Cell Lymphoma:

- Celiac Enteropathy Associated  

- T-Cell Lymphoma with tissue eosinophilia

- Other T-Cell Lymphoma with Tissue Eosinophilia

- Other T- Cell Lymphoma
 

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 .

Example:   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.

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.

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 and 79a : positive

CD10: positive,

CD5 and 23:  negative

Ki67 : more than 85% cell are positive.

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

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).

 

Further reading:

Critical evaluation of Bcl-6 protein expression in diffuse large B- cell lymphoma of the stomach and small intestine. 

Primary follicular lymphoma of the gastrointestinal tract: a study of 25 cases and a literature review. 

Primary follicular lymphoma of the gastrointestinal: a clinical and pathologic study of 26 cases. 

Problems in biopsy differential diagnosis in lymphomas of the small and large intestine.

Classification of intestinal T- cell neoplasms and their differential diagnosis. 

Primary lymphoma of the small intestine. A clinico-pathologic study of 119 cases. 

Gastrointestinal lymphomas of T and B cell types.  

Extranodal lymphomas: the MALT concept. 

 

Lymphoid hyperplasia of small intestine is a reactive process:

- 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.

- Giardia lamblia infection is noted in some patients with lymphoid hyperplasia.

- Differential Diagnosis : 

Malignant lymphoma: Immunohistochemistry is performed to confirm the diagnosis.

 

Lymphoid hyperplasia of small intestine is a reactive process:

- 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.

- Giardia lamblia infection is noted in some patients with lymphoid hyperplasia.

- Differential Diagnosis : 

Malignant lymphoma: Immunohistochemistry is performed to confirm the diagnosis.

 

Further reading:


Lymphoid hyperplasia of the intestine in children. 15 cases. 

Florid reactive lymphoid hyperplasia of the terminal ileum in adults. A condition bearing a close resemblance to low-grade malignant lymphoma.  

Nodular lymphoid hyperplasia of the intestine tract in infancy and childhood. 

Nodular lymphoid hyperplasia of the bowel in primary hypogammaglobulinaemia: a study of in vivo and in vitro lymphocyte function 

 

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Pathology of Small Intestine - Home Page

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

 

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