Mediterranean lymphoma. Diffuse immunoblastic entero-mesenteric sarcoma without IgA abnormality
Galian, A.; Scotto, J.; Rouffy, J.; James, J.M.; Lenormand, Y.; Rambaud, J.C.
Gastroenterologie Clinique et Biologique 1(1): 49-58
1977
ISSN/ISBN: 0399-8320 PMID: 873105 Document Number: 110355
An Algerian man, 22 yr old, was admitted for continuous abdominal pain, chronic and copious diarrhea, vomiting and poor general condition. The digestive symptoms started 6 yr ago. Two years before admission, clinical and biological signs of hypoparathyroidism were rapidly cured by vitamin D2. Physical examination showed cachexia, leg edema, pleural and pericardial effusions, a segmented abdominal mass and enlarged axillary nodes. Biological investigations revealed a malabsorption syndrome and no serum immunoglobulin abnormality. The patient died within a few weeks. Light microscopy and EM study showed immunoblastic cellular proliferation which infiltrated the whole wall of the entire small bowel and destroyed the normal axillary and mesenteric lymph node architecture. Portal triads and serous effusions also contained immunoblastic cells. A few cells showed a trend toward plasmocytic differentiation, but no intracytoplasmic immunoglobulin was observed by immunofluorescence techniques. Besides .alpha.-chain disease, Mediterranean lymphomas include other histological types of diffuse intestinal sarcomas.