Questions and Answers
Which of the following sites is least commonly associated with MALT lymphoma?
Which of the following cytogenetic abnormalities is most frequently found in MALT lymphoma?
What is the primary pathological feature of MALT lymphoma?
Which type of infection is most commonly associated with Alpha heavy chain disease?
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What characterizes the early-stage response to treatment in IPSID?
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Study Notes
MALT Lymphoma
- MALT (mucosa-associated lymphoid tumor) lymphoma is classified as an extranodal marginal zone lymphoma.
- Most commonly manifests in the stomach; other frequent locations include skin, salivary glands, small bowel, ocular adnexa, and synovium.
- Associated with chronic inflammation from autoimmune diseases (e.g., Sjögren's syndrome, Hashimoto's thyroiditis) or chronic infections (e.g., H. pylori, Borrelia burgdorferi, C. psittaci, C. jejuni, hepatitis C virus).
- Characterized by lymphoepithelial lesions due to neoplastic lymphocytes invading mucosal glands and crypts.
- Tumor cells display markers CD19, CD20, and CD79a but are negative for CD5 and CD10.
Cytogenetic Abnormalities in MALT Lymphoma
- Common recurrent cytogenetic abnormalities include:
- t(11;18), t(14;18), t(1;14), t(3;14), trisomy 8.
- t(11;18) is the most prevalent, found in up to 50% of MALT lymphoma cases.
Clinical Presentation and Treatment
- MALT lymphomas typically present at stage I or II, contrasting with other indolent B-cell lymphomas.
- Radiation therapy can be curative in early-stage cases.
- Patients may also benefit from antibiotic treatment targeting chronic infections linked to lymphoma onset.
Alpha Heavy Chain Disease / IPSID
- Alpha heavy chain disease/Immunoproliferative Small Intestinal Disease (IPSID) is an infectious pathogen-associated human lymphoma, mainly associated with Campylobacter jejuni.
- Primarily affects the proximal small intestine, leading to symptoms like malabsorption, diarrhea, and abdominal pain.
- Associated with excessive plasma cell differentiation, producing truncated alpha heavy chain proteins that lack light chains.
- Early-stage IPSID shows a partial response to antibiotics, with a 30-70% rate of complete remission.
- Most untreated IPSID cases progress to lymphoplasmacytic and immunoblastic lymphoma.
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Description
This quiz covers MALT lymphoma, an extranodal marginal zone lymphoma. It explores its common locations, associated autoimmune diseases, cytogenetic abnormalities, and clinical presentation. Ideal for medical students and professionals seeking to deepen their understanding of this specific lymphoma type.
