Abstract
Ultrastructural and immunohistochemical studies of the small intestine are described in a Japanese patient with alpha heavy chain disease who had a history of colonic ulcers. Endoscopic examinations revealed multiple gastric erosion, duodenal ulcer, and a thickened, cobblestone-like pattern composed of small nodules in the duodenum and jejunum, which was similar in appearance to Crohn's disease. An electron microscopic study showed that the numerous, infiltrated cells in the jejunal lamina propria were matured plasma cells with atypical structure of the organelles. These plasma cells had alpha heavy chain protein devoid of light chain. Although secretory component was demonstrated normally in the epithelial cells by immunofluorescent methods, no association of this component with alpha heavy chain protein could be observed in any of the plasma and epithelial cells of this case. These facts suggest the absence of secretory alpha chain or secretory IgA, and a deficiency of the mucosal secretory immune system in this patient.
Full text
PDF





Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Al-Bahrani Z., Al-Saleem T., Al-Mondhiry H., Bakir F., Yahia H., Taha I., King J. Alpha heavy chain disease (report of 18 cases from Iraq). Gut. 1978 Jul;19(7):627–631. doi: 10.1136/gut.19.7.627. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Bloth B., Svehag S. E. Further studies on the ultrastructure of dimeric IgA of human origin. J Exp Med. 1971 May 1;133(5):1035–1042. doi: 10.1084/jem.133.5.1035. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Brandtzaeg P. Mucosal and glandular distribution of immunoglobulin components. Immunohistochemistry with a cold ethanol-fixation technique. Immunology. 1974 Jun;26(6):1101–1114. [PMC free article] [PubMed] [Google Scholar]
- Brown W. R., Isobe K., Nakane P. K., Pacini B. Studies on translocation of immunoglobulins across intestinal epithelium. IV. Evidence for binding of IgA and IgM to secretory component in intestinal epithelium. Gastroenterology. 1977 Dec;73(6):1333–1339. [PubMed] [Google Scholar]
- Cohen H. J., Gonzalvo A., Krook J., Thompson T. T., Kremer W. B. New presentation of alpha heavy chain disease: North American polypoid gastrointestinal lymphoma. Clinical and cellular studies. Cancer. 1978 Mar;41(3):1161–1169. doi: 10.1002/1097-0142(197803)41:3<1161::aid-cncr2820410354>3.0.co;2-j. [DOI] [PubMed] [Google Scholar]
- 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]
- Doe W. F., Henry K., Doyle F. H. Radiological and histological findings in six patients with alpha-chain disease. Br J Radiol. 1976 Jan;49(577):3–11. doi: 10.1259/0007-1285-49-577-3. [DOI] [PubMed] [Google Scholar]
- Gafter U., Kessler E., Shabtay F., Shaked P., Djaldetti M. Abnormal chromosomal marker (D14 q+) in a patient with alpha heavy chain disease. J Clin Pathol. 1980 Feb;33(2):136–144. doi: 10.1136/jcp.33.2.136. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Galian A., Lecestre M. J., Scotto J., Bognel C., Matuchansky C., Rambaud J. C. Pathological study of alpha-chain disease, with special emphasis on evolution. Cancer. 1977 May;39(5):2081–2101. doi: 10.1002/1097-0142(197705)39:5<2081::aid-cncr2820390526>3.0.co;2-e. [DOI] [PubMed] [Google Scholar]
- Haghighi P., Tabei Z., Kharazmi A., Gerami S., Abadi P., Haghshenass M. Immunoperoxidase study in alpha-chain disease. Arch Pathol Lab Med. 1978 Nov;102(11):555–557. [PubMed] [Google Scholar]
- Haghshenass M., Haghighi P., Abadi P., Kharazmi A., Gerami C., Nasr K. Alpha heavy-chain disease in southern Iran. Am J Dig Dis. 1977 Oct;22(10):866–873. doi: 10.1007/BF01076161. [DOI] [PubMed] [Google Scholar]
- 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]
- Plesnicar S., Sumi-Kriznik T., Golouh R. Abdominal lymphoma with alpha-heavy chain disease. Isr J Med Sci. 1975 Aug;11(8):832–837. [PubMed] [Google Scholar]
- 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]
- Ramot B., Levanon M., Hahn Y., Lahat N., Moroz C. The mutual clonal origin of the lymphoplasmocytic and lymphoma cell in alpha-heavy chain disease. Clin Exp Immunol. 1977 Mar;27(3):440–445. [PMC free article] [PubMed] [Google Scholar]
- 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]
- Seligmann M. Immunochemical, clical, and pathological features of alpha-chain disease. Arch Intern Med. 1975 Jan;135(1):78–82. [PubMed] [Google Scholar]
- Seligmann M., Mihaesco E., Hurez D., Mihaesco C., Preud'homme J. L., Rambaud J. C. Immunochemical studies in four cases of alpha chain disease. J Clin Invest. 1969 Dec;48(12):2374–2389. doi: 10.1172/JCI106204. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Shulman G., York E. L., Grieve S. Alpha-chain disease in a non-Mediterranean climate. A case report. S Afr Med J. 1975 Dec 13;49(53):2183–2186. [PubMed] [Google Scholar]
- 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]
- 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]
- 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]