Skip to main content
PMC home page
Gut logoLink to Gut
. 1972 Dec;13(12):947–957. doi: 10.1136/gut.13.12.947

Five cases of alpha chain disease

William F Doe, K Henry, J R Hobbs, F Avery Jones, C E Dent, C C Booth
PMCID: PMC1412503  PMID: 4119805

Abstract

Five patients suffering from alpha chain disease are described. Clinically the patients presented with clubbing and the symptoms of malabsorption. There was a characteristic, predominantly plasma cell infiltrate of the wall of the small intestine. Spread of the plasmacytosis beyond the small intestine to bone marrow (1), peripheral blood (1), and probably the nasopharyngeal lymphoid tissue (1) is described. Fragments of the heavy chain of IgA (alpha chain) were found in serum (5), urine (3), jejunal fluid (2), and saliva (1). The jejunal biopsy of one patient was shown to synthesize free alpha chain in tissue culture. A new and simple immunoselection technique for the identification of free alpha chain is described. Marked clinical remissions were achieved in two patients treated with intermittent cytotoxic and steroid therapy, and in a third patient who received intermittent cytotoxic therapy and tetracycline.

Full text

PDF
953

Images in this article

950Fig. 1
on p.950
950Fig. 2
on p.950
951Fig. 3
on p.951
952Fig. 4
on p.952
953Fig. 5
on p.953
954Fig. 6
on p.954
954Fig. 7
on p.954
955Fig. 8
on p.955

Selected References

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

  1. Ballard H. S., Hamilton L. M., Marcus A. J., Illes C. H. A new variant of heavy-chain disease (mu-chain disease). N Engl J Med. 1970 May 7;282(19):1060–1062. doi: 10.1056/NEJM197005072821902. [DOI] [PubMed] [Google Scholar]
  2. Dent C. E., Norris T. S., Smith R., Sutton R. A., Temperley J. M. Steatorrhoea with striking increase of plasma-alkaline-phosphatase of intestinal origin. Lancet. 1968 Jun 22;1(7556):1333–1336. doi: 10.1016/s0140-6736(68)92035-7. [DOI] [PubMed] [Google Scholar]
  3. FRANKLIN E. C., LOWENSTEIN J., BIGELOW B., MELTZER M. HEAVY CHAIN DISEASE- A NEW DISORDER OF SERUM GAMMA-GLOBULINS : REPORT OF THE FIRST CASE. Am J Med. 1964 Sep;37:332–350. doi: 10.1016/0002-9343(64)90191-3. [DOI] [PubMed] [Google Scholar]
  4. Irunberry J., Benallègue A., Illoul G., Timsit G., Abbadi M., Benabdallah S., Boucekkine T., Ould-Aoudia J. P., Colonna P. Trois cas de maladie des chaînes alpha observés en Algérie. Nouv Rev Fr Hematol. 1970 Sep-Oct;10(5):609–616. [PubMed] [Google Scholar]
  5. Laroche C., Seligmann M., Merillon H., Turpin G., Marche C., Cerf M., Lemaigre G., Forest M., Hurez D. Nouvelle observation d'une maladie des chaines lourdes alpha au cours d'un lymphome abdominal de type méditerranéen avec tuberculose isolée des ganglions mésentériques et pelvi-spondylite. Presse Med. 1970 Jan 10;78(2):55–59. [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. 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]
  9. Rádl J. Light chain typing of immunoglobulins in small samples of biological material. Immunology. 1970 Jul;19(1):137–149. [PMC free article] [PubMed] [Google Scholar]
  10. Scotto J., Stralin H., Caroli J. Ultrastructural study of two cases of alpha-chain disease. Gut. 1970 Sep;11(9):782–788. doi: 10.1136/gut.11.9.782. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. 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]
  12. 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]
  13. 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]
  14. Zlotnick A., Levy M. Alpha heavy chain disease. A variant of Mediterranean lymphoma. Arch Intern Med. 1971 Sep;128(3):432–436. doi: 10.1001/archinte.128.3.432. [DOI] [PubMed] [Google Scholar]

Articles from Gut are provided here courtesy of BMJ Publishing Group

RESOURCES