Skip to main content
PMC home page
Gut logoLink to Gut
. 1978 Jul;19(7):627–631. doi: 10.1136/gut.19.7.627

Alpha heavy chain disease (report of 18 cases from Iraq).

Z Al-Bahrani, T Al-Saleem, H Al-Mondhiry, F Bakir, H Yahia, I Taha, J King
PMCID: PMC1412087  PMID: 98395

Abstract

The clinical and pathological features of 18 new patients with alpha heavy chain disease seen at two referral centres in Baghdad, Iraq, are described. The series included 14 males and four females ranging in age from 14 to 47 years. Almost all patients presented because of long-standing abdominal pain and diarrhoea. The tissue diagnosis and extent of the disease were established at laparotomy in most patients. Peroral jejunal biospy was used in a number of patients, mainly for follow-up. The serological abnormality was confirmed by immunoselection technique. Most of the patients had extensive thickening of the bowel wall and/or tumour masses of the small intestine and mesenteric nodes. Histopathological sections showed muscularis. Preliminary results of the treatment, including two long remissions, are reported. In general, our observations agree with those made by other authors, mostly from the Middle East and Africa. We believe that a high index of clinical suspicion, routine use of the immunoselection, and recognition of the early pathological changes may hopefully lead to the detection of more cases before the frank neoplastic phase of the disease.

Full text

PDF
627

Images in this article

628Figure
on p.628

Selected References

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

  1. Doe W. F. Alpha chain disease clinicopathological features and relationship to so-called Mediterranean lymphoma. Br J Cancer Suppl. 1975 Mar;2:350–355. [PMC free article] [PubMed] [Google Scholar]
  2. 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]
  3. Guardia J., Rubies-Prat J., Gallart M. T., Moragas A., Martinez-Vazquez J. M., Bacardi R., Vilaseca J. The evolution of alpha heavy chain disease. Am J Med. 1976 Apr;60(4):596–602. doi: 10.1016/0002-9343(76)90729-4. [DOI] [PubMed] [Google Scholar]
  4. Kharazmi A., Haghighi P., Haghshenas M., Nasr K., Abadi P., Rezai H. R. Alpha-chain disease and its association with intestinal lymphoma. Clin Exp Immunol. 1976 Oct;26(1):124–128. [PMC free article] [PubMed] [Google Scholar]
  5. Lewin K. J., Kahn L. B., Novis B. H. Primary intestinal lymphoma of "Western" and "Mediterranean" type, alpha chain disease and massive plasma cell infiltration: a comparative study of 37 cases. Cancer. 1976 Dec;38(6):2511–2528. doi: 10.1002/1097-0142(197612)38:6<2511::aid-cncr2820380641>3.0.co;2-u. [DOI] [PubMed] [Google Scholar]
  6. Manousos O. N., Economidou J. C., Georgiadou D. E., Pratsika-Ougourloglou K. G., Hadziyannis S. J., Merikas G. E., Henry K., Doe W. F. Alpha-chain disease with clinical, immunological, and histological recovery. Br Med J. 1974 May 25;2(5916):409–412. doi: 10.1136/bmj.2.5916.409. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. 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]
  8. Rambaud J. C., Seligmann M. Alpha-chain disease. Clin Gastroenterol. 1976 May;5(2):341–358. [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. 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]
  11. 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]
  12. Skinner J. M., Manousos O. N., Economidou J., Nicolaou A., Merikas G. Alpha-chain disease with localized plasmacytoma of the intestine. Immunoperoxidase study. Clin Exp Immunol. 1976 Jul;25(1):112–116. [PMC free article] [PubMed] [Google Scholar]
  13. Tabbane S., Tabbane F., Cammoun M., Mourali N. Mediterranean lymphomas with alpha heavy chain monoclonal gammopathy. Cancer. 1976 Nov;38(5):1989–1996. doi: 10.1002/1097-0142(197611)38:5<1989::aid-cncr2820380521>3.0.co;2-y. [DOI] [PubMed] [Google Scholar]

Articles from Gut are provided here courtesy of BMJ Publishing Group

RESOURCES