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. 1976 Oct;26(1):124–128.

Alpha-chain disease and its association with intestinal lymphoma.

A Kharazmi, P Haghighi, M Haghshenas, K Nasr, P Abadi, H R Rezai
PMCID: PMC1540810  PMID: 826361

Abstract

Twenty-seven intestinal lymphoma patients were studied. Abnormal alpha-chain immunoglobulin was detected in the sera of seven of these patients. The alpha-chain disease patients were from the rural areas of Southern Iran. They were of low socio-economic status and their age ranged from 15-44 years. Predominant clinical features were malabsorption, diarrhoea , abdominal pain, vomiting, and weight loss. Infiltration of mucosa of the small intestine with plasma cells and also distortion and flattening of the villi were common histopathologic characteristics of these patients. Involvement of mesenteric lymph nodes with infiltration of tumour cells was observed in a number of cases. Protein studies revealed no significant differences between the serum immunoglobulin levels of these patients and normal values. Immunoelectrophoresis using monospecific antiserum against H-chain of human IgA demonstrated the abnormal precipitin band of alpha-chain disease protein.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Doe W. F., Henry K., Hobbs J. R., Jones F. A., Dent C. E., Booth C. C. Five cases of alpha chain disease. Gut. 1972 Dec;13(12):947–957. doi: 10.1136/gut.13.12.947. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Dutz W., Asvadi S., Sadri S., Kohout E. Intestinal lymphoma and sprue: a systematic approach. Gut. 1971 Oct;12(10):804–810. doi: 10.1136/gut.12.10.804. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Laroche C., Mérillon H., Turpin G. Le lymphome méditerranéen. Presse Med. 1970 Jan 10;78(2):53–54. [PubMed] [Google Scholar]
  4. Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
  5. Nasr K., Haghighi P., Bakhshandeh K., Haghshenas M. Primary lymphoma of the upper small intestine. Gut. 1970 Aug;11(8):673–678. doi: 10.1136/gut.11.8.673. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Novis B. H., Bank S., Marks I. N., Selzer G., Kahn L., Sealy R. Abdominal lymphoma presenting with malabsorption. Q J Med. 1971 Oct;40(160):521–540. [PubMed] [Google Scholar]
  7. Rambaud J. C., Bognel C., Prost A., Bernier J. J., Le Quintrec Y., Lambling A., Danon F., Hurez D., Seligmann M. Clinico-pathological study of a patient with "Mediterranean" type of abdominal lymphoma and a new type of IgA abnormality ("alpha chain disease"). Digestion. 1968;1(6):321–336. doi: 10.1159/000196874. [DOI] [PubMed] [Google Scholar]
  8. Rambaud J. C., Matuchansky C. Alpha-chain disease. Pathogenesis and relation to Mediterranean lymphoma. Lancet. 1973 Jun 23;1(7817):1430–1432. doi: 10.1016/s0140-6736(73)91752-2. [DOI] [PubMed] [Google Scholar]
  9. Ramot B., Hulu N. Primary intestinal lymphoma and its relation to alpha heavy chain disease. Br J Cancer Suppl. 1975 Mar;2:343–349. [PMC free article] [PubMed] [Google Scholar]
  10. Ramot B. Malabsorption due to lymphomatous disease. Annu Rev Med. 1971;22:19–24. doi: 10.1146/annurev.me.22.020171.000315. [DOI] [PubMed] [Google Scholar]
  11. Rappaport H., Ramot B., Hulu N., Park J. K. The pathology of so-called Mediterranean abdominal lymphoma with malabsorption. Cancer. 1972 Jun;29(6):1502–1511. doi: 10.1002/1097-0142(197206)29:6<1502::aid-cncr2820290614>3.0.co;2-o. [DOI] [PubMed] [Google Scholar]
  12. Seligmann M., Danon F., Hurez D., Mihaesco E., Preud'homme J. L. Alpha-chain disease: a new immunoglobulin abnormality. Science. 1968 Dec 20;162(3860):1396–1397. doi: 10.1126/science.162.3860.1396. [DOI] [PubMed] [Google Scholar]
  13. Seligmann M., Mihaesco E., Frangione B. Alpha chain disease. Ann N Y Acad Sci. 1971 Dec 31;190:487–500. doi: 10.1111/j.1749-6632.1971.tb13558.x. [DOI] [PubMed] [Google Scholar]
  14. Shahid M. J., Alami S. Y., Nassar V. H., Balikian J. B., Salem A. A. Primary intestinal lymphoma with paraproteinemia. Cancer. 1975 Mar;35(3):848–858. doi: 10.1002/1097-0142(197503)35:3<848::aid-cncr2820350342>3.0.co;2-n. [DOI] [PubMed] [Google Scholar]
  15. Stoop J. W., Ballieux R. E., Hijmans W., Zegers B. J. Alpha-chain disease with involvement of the respiratory tract in a Dutch child. Clin Exp Immunol. 1971 Nov;9(5):625–635. [PMC free article] [PubMed] [Google Scholar]

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