IMMUNOPROLIFERATIVE SMALL INTESTINAL DISEASE
 
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    IMMUNOPROLIFERATIVE SMALL INTESTINAL DISEASE

     

    DEFINITION:

    An IgA lymphoproliferative disorder of the small bowel resulting in diarrhea, malabsorption, and eventually a B cell lymphoma.

    EPIDEMIOLOGY:

    • incidence: ?
    • age of onset:
      • 10 - 30 years
    • risk factors:
      • environmental:
        • endemic - Mediterranean basin, Mideast, Far East, Africa
        • sporadic - Europe, North & South America, immigrants from developing countries, native Indians
        • poverty
      • frequent episodes of gastroenteritis:
        • ? Giardia Lamblia

    PATHOGENESIS:

    1. Etiology

    • IgA lymphoproliferation (due to an unknown antigen - ? Giardia) within the mucosa causes direct damage to the enterocytes -> malabsorption

    CLINICAL FEATURES:

    1. Gastrointestinal Manifestations

    1. Initially

    • intermittent diarrhea and abdominal pain

    2. Later

    • persistent chronic diarrhea
    • malabsorption
    • weight loss and growth failure
    • digital clubbing

    3. Complications

    • high-grade small intestinal B cell lymphoma

    INVESTIGATIONS:

    1. For Malabsorption

    • see "Malabsorptive Disorders"

    2. Serum

    • IgA (alpha heavy chain paraprotein) may act as a serum marker

    3. Biopsy - Duodenum or Jejunum

    • biopsies at multiple sites

    1. Early Lesions

    • thickened patchy mucosal folds
    • duodenal or jejunal nodularity
    • lymphoplasmacytic infiltrates

    2. Prelymphomatous Stage

    • diffuse lymphohistiocytic nodules
    • mesenteric lymph node involvement
    • presence of Reed-Sternberg-like cells

    3. Lymphomas

    • immunoblastic lymphoma

    Note: The pathology represents an IgA lymphoproliferative disorder progressing to a B cell lymphoma.

    4. Stool

    • Giardia Lamblia may be identified

    MANAGEMENT:

    1. Early Stage

    • tetracycline for 6 months
    • usually a good outcome

    2. Prelymphomatous Stage

    • cyclophosphamide +/- prednisone & tetracycline
    • variable but usually a poor outcome

    3. Lymphomas

    • cyclophosphamide, doxorubicin, teniposide, vinblastine, prednisone +/- bleomycin

     

     

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