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. 1975 Mar;2:356–361.

Alpha chain disease: immunoglobulin abnormalities, pathogenesis and current concepts.

M Seligmann
PMCID: PMC2149602  PMID: 810152

Abstract

The laboratory findings upon which the diagnosis of alpha chain disease relies and the main results of immunochemical, structural and biosynthetic studies of the pathological immunoglobulin are reviewed briefly. The pathogenesis of the disease is discussed in view of its possibly non-malignant nature at the early stage and of its peculiar geographic distribution, suggesting the triggering role of an intestinal micro-organism.

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Selected References

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

  1. Bognel J. C., Rambaud J. C., Modigliani R., Matuchansky C., Bognel C., Bernier J. J., Scotto J., Hautefeuille P., Mihaesco E., Hurez D. Etude clinique, anatomo-pathologique et immunochemique d'un nouveau cas de maladie des chaines alpha suivi pendant cinq ans. Rev Eur Etud Clin Biol. 1972 Apr;17(4):362–374. [PubMed] [Google Scholar]
  2. Buxbaum J. N., Preud'homme J. L. Alpha and gamma heavy chain diseases in man: intracellular origin of the aberrant polypeptides. J Immunol. 1972 Nov;109(5):1131–1137. [PubMed] [Google Scholar]
  3. 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]
  4. Faux J. A., Crain J. D., Rosen F. S., Merler E. An alpha heavy chain abnormality in a child with hypogammaglobulinemia. Clin Immunol Immunopathol. 1973 Jan;1(2):282–290. doi: 10.1016/0090-1229(73)90028-7. [DOI] [PubMed] [Google Scholar]
  5. Frangione B., Franklin E. C. Heavy chain diseases: clinical features and molecular significance of the disordered immunoglobulin structure. Semin Hematol. 1973 Jan;10(1):53–64. [PubMed] [Google Scholar]
  6. Frangione B., Milstein C. Partial deletion in the heavy chain disease protein ZUC. Nature. 1969 Nov 8;224(5219):597–599. doi: 10.1038/224597a0. [DOI] [PubMed] [Google Scholar]
  7. Natori S., Garen A. Molecular heterogeneity in the amino-terminal region of alkaline phosphatase. J Mol Biol. 1970 May 14;49(3):577–588. doi: 10.1016/0022-2836(70)90282-2. [DOI] [PubMed] [Google Scholar]
  8. RAMOT B., SHAHIN N., BUBIS J. J. MALABSORPTION SYNDROME IN LYMPHOMA OF SMALL INTESTINE. A STUDY OF 13 CASES. Isr J Med Sci. 1965 Mar;1:221–226. [PubMed] [Google Scholar]
  9. 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]
  10. 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]
  11. Rambaud J. C., Matuchansky C., Bognel J. C., Bognel C., Bernier J. J., Scotto J., Perol C., Ferrier J. P., Mihaesco E., Hurez D. Nouveau cas de maladie des chaînes alpha chez un Eurasien. Ann Med Interne (Paris) 1970 Feb;121(2):135–148. [PubMed] [Google Scholar]
  12. 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]
  13. Rogé J., Druet P., Marche C. Lymphome méditerranéen avec maladie des chaînes alpha. Triple rémission clinique, anatomique et immunologique. Pathol Biol (Paris) 1970 Oct;18(19):851–858. [PubMed] [Google Scholar]
  14. 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]
  15. Seligmann M. Heavy chain diseases. Rev Eur Etud Clin Biol. 1972 Apr;17(4):349–355. [PubMed] [Google Scholar]
  16. 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]
  17. 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]
  18. 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]
  19. Wolfenstein-Todel C., Mihaesco E., Frangione B. "Alpha chain disease" protein def: internal deletion of a human immunoglobulin A1 heavy chain. Proc Natl Acad Sci U S A. 1974 Mar;71(3):974–978. doi: 10.1073/pnas.71.3.974. [DOI] [PMC free article] [PubMed] [Google Scholar]

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