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[Alpha-heavy chain disease--Mediterranean lymphoma]
H P Kaufmann1, W Schmitt, H J Seib
1I. Med. Abteilung, Städt. Krankenhaus München-Neuperlach, Akademisches Lehrkrankenhaus.
Leber, Magen, Darm
|February 1, 1994
Summary
A rare alpha-Heavy Chain Disease, also known as immunoproliferative small intestinal disease (IPSID), was diagnosed in a young man presenting with severe diarrhea and weight loss. Antibiotic treatment showed no effect on the disease progression.
Area of Science:
- Gastroenterology
- Hematology
- Oncology
Background:
- Immunoproliferative small intestinal disease (IPSID), or Mediterranean lymphoma, is a rare condition characterized by a malignant lymphoma of the small intestine.
- It is associated with a monoclonal immunoglobulin A (IgA) gammopathy.
Observation:
- A 28-year-old man from the Emirates presented with a 6-month history of severe diarrhea and a 30 kg weight loss.
- Endoscopic examination revealed lymphoma affecting the entire small intestine.
Findings:
- Biopsies, immunohistochemistry, and immunologic tests confirmed a malignant MALT-lymphoma of B-cell type, specifically alpha-Heavy Chain Disease (IPSID).
- The disease was staged as A with progression to stage B.
- Treatment with ciprofloxacin and metronidazole did not influence the disease course.
Implications:
- This case highlights the diagnostic challenges and aggressive nature of IPSID.
- Further research into effective therapeutic strategies for IPSID is warranted.
- Early diagnosis and understanding the pathophysiology are crucial for managing this rare lymphoma.