Advertisement
Research Article| Volume 6, ISSUE 3, P477-489, September 1986

Alpha-Heavy Chain Disease

  • Parviz Haghighi
    Correspondence
    Corresponding Author: Department of Pathology, Veterans Administration Hospital, 3350 La Jolla Village Drive, La Jolla, California 92037.
    Affiliations
    Clinical Professor, Department of Pathology, University of California at San Diego, and Staff Pathologist, Veterans Administration Medical Center, La Jolla, California
    Search for articles by this author
  • Paul L. Wolf
    Affiliations
    Professor of Pathology, University of California Medical Center, San Diego, California
    Search for articles by this author
DOI:https://doi.org/10.1016/S0272-2712(18)30794-7
Alpha-Heavy Chain Disease
Previous ArticleThe Cerebrospinal Fluid Proteins in Multiple Sclerosis
Next ArticleThe Clinical Use of Alkaline Phosphatase Enzymes
      This paper is only available as a PDF. To read, Please Download here.
      A review of alpha-heavy chain disease (AHCD) emphasizing its histopathology and associated lesions is presented. Unusual clinicopathologic presentations and modern concepts regarding histogenesis of AHCD in the light of recent immunohistochemical findings are discussed. Increasingly reported cases of Burkitt’s (or Burkitt-type) lymphoma involving distal small intestine or the ileocecal region in children are noted.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribers receive full online access to your subscription and archive of back issues up to and including 2002.

      Content published before 2002 is available via pay-per-view purchase only.

      Subscribe:

      Subscribe to Clinics in Laboratory Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Register: Create an account
      Institutional Access: Sign in to ScienceDirect

      References

        • Al-Bahrani Z.R.
        • Al-Mondhiry H.
        • Bakir F.
        • Al-Saleem T.
        Clinical and pathologic subtypes of primary intestinal lymphoma.
        Cancer. 1983; 52: 1666
        • Al-Saleem T.I.
        Evidence of acquired immune deficiencies in Mediterranean lymphoma: A possible aetiological link.
        Lancet. 1978; 2: 709
        • Aozosa K.
        • Tsumimoto M.
        • Inoue A.
        • et al.
        Primary gastrointestinal lymphoma: A clinicopathologic study of 102 patients.
        Oncology. 1985; 42: 97
        • Asselah F.
        • Slavin G.
        • Sowter G.
        • Asselah H.
        Immunoproliferative small intestinal disease in Algerians. I. Light microscopic and immunochemical studies.
        Cancer. 1983; 52: 227
        • Baklein K.
        • Fausa O.
        • Brandtzaeg P.
        • et al.
        Malabsorption, villous atrophy and excessive serum IgA in a patient with unusual intestinal immunocyte infiltration.
        Scand. J. Gastroenterol. 1977; 12: 421
        • Bender S.W.
        • Danon F.
        • Preud’homme J.L.
        • et al.
        Gamma heavy chain disease simulating alpha-chain diseaase.
        Gut. 1978; 19: 1148

      7. Blackshaw, A. J.: Non-Hodgkin’s lymphomas of the gut. In Wright, R. (ed): Recent Advances in Gastrointestinal Pathology. Clin. Gastroenterol. Suppl. I, 1980, pp. 213–240.

        • Brandtzaeg P.
        • Baklein K.
        Characterization of the IgA immunocyte population and its products in a patient with excessive intestinal formation of IgA.
        Clin. Exp. Immunol. 1977; 30: 77
        • Buxbaum J.
        • Preud’homme J.L.
        Alpha and gamma heavy chain diseases in man: Intracellular origin of the aberrant polypeptides.
        J. Immunol. 1972; 109: 1131
        • Chantar C.
        • Escartin P.
        • Plaza A.G.
        • et al.
        Diffuse plasma cell infiltration of the small intestine with malabsorption associated to IgA monoclonal gammopathy.
        Cancer. 1974; 34: 1620
        • Cohen H.J.
        • Gonzalvo A.
        • Krook J.
        • et al.
        New presentation of alpha heavy chain disease: North American polypoid gastrointestinal lymphoma. Clinical and cellular studies.
        Cancer. 1978; 41: 1161
        • Collins J.
        • Katon R.
        • Harty-Golder B.
        Burkitt’s lymphoma presenting with gastroduodenal involvement.
        Gastroenterology. 1983; 85: 425
        • Contreary K.
        • Nance F.C.
        • Becker W.F.
        Primary lymphoma of the gastrointestinal tract.
        Ann. Surg. 1980; 191: 593
        • Cowan N.J.
        • Secher D.C.
        • Milstein C.
        Intracellular immunoglobulin chain synthesis in nonsecreting variants of a mouse myeloma: Detection of inactive light chain messenger RNA.
        J. Mol. Biol. 1974; 90: 691
        • Crow J.
        • Asselah F.
        Immunoproliferative small intestinal disease in Algerians. II. Ultrastructural studies in alpha-chain disease.
        Cancer. 1984; 54: 1908
        • Dackowski W.
        • Morrison S.
        Two alpha heavy chain disease proteins with different genomic deletions demonstrate that non-expressed alpha-heavy chain genes contain methylated bases.
        Proc. Natl. Acad. Sci. USA. 1981; 78: 7091
        • Daum G.S.
        • Lipman M.
        • Woda B.A.
        Dendritic cell phenotype in localized malignant histiocytosis of the small intestine.
        Arch. Pathol. Lab. Med. 1985; 109: 647
        • Dawson I.M.P.
        • Cornes J.S.
        • Morson B.C.
        Primary malignant lymphoid tumors of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis.
        Br. J. Surg. 1961; 49: 80
        • Doe W.F.
        • Danon F.
        • Seligmann M.
        Immunodiagnosis of alpha-chain disease.
        Exp. Immunol. 1979; 36: 189
        • Dorrington K.J.
        • Mihaesco E.
        • Seligmann M.
        The molecular size of three alphachain disease proteins.
        Biochim. Biophys. Acta. 1970; 221: 647
        • Dragosics B.
        • Bauer P.
        • Radaszkiewicz J.
        Primary gastrointestinal non-Hodgkin’s lymphomas. A retrospective clinicopathologic study of 150 cases.
        Cancer. 1985; 55: 1060
        • Economidou J.C.
        • Monousos O.N.
        • Katsaros D.
        Alpha-chain disease causing kaliopenic nephropathy and fatal intestinal perforations.
        Dig. Dis. Sci. 1976; 21: 577
        • Eidelman S.
        • Parkins R.A.
        • Rubin E.
        Abdominal lymphoma presenting as malabsorption: A clinicopathologic study of nine cases in Israel and review of the literature.
        Medicine (Baltimore). 1966; 45: 111
        • Fairley N.H.
        • Mackie F.P.
        The clinical and biochemical syndrome in lymphadenoma and allied diseases involving the mesenteric lymph glands.
        Br. Med. J. 1937; 1: 375
        • Filippa D.A.
        • Lieberman P.H.
        • Weingrad D.N.
        • et al.
        Primary lymphomas of the gastrointestinal tract.
        Am. J. Surg., Pathol. 1983; 7: 363
        • Foucar K.
        • Foucar E.
        • Mitros F.
        • et al.
        Epitheliotropic lymphoma of the small bowel.
        Cancer. 1984; 54: 54
        • Freeman H.J.
        • Weinstein W.M.
        • Shnitka J.K.
        • et al.
        Primary abdominal lymphoma. Presenting manifestation of celiac sprue or complicating dermatitis herpetiformis.
        Am. J. Med. 1977; 63: 585
        • Gonzales-Vitale J.C.
        • Gomez L.G.
        • Goldblum R.M.
        • et al.
        Immunoblastic lymphoma of small intestine complicating late-onset immunodeficiency.
        Cancer. 1982; 49: 445
        • Gray G.M.
        • Rosenberg S.A.
        • Cooper A.D.
        • et al.
        Lymphomas involving the gastrointestinal tract.
        Gastroenterology. 1982; 32: 143
        • Guardia J.
        • Rubies-Part J.
        • Gallant M.
        • et al.
        The evolution of alpha-heavy chain disease.
        Am. J. Med. 1976; 60: 596
        • Guardia J.
        • Mirada A.
        • Moragas A.
        • et al.
        Alpha-chain disease of the stomach.
        Hepatogastroenterology. 1980; 28: 238

      32. Haghighi, P.: Unpublished data.

        • Haghighi P.
        • Nasr K.
        Primary upper small intestinal lymphoma (so-called Mediterranean lymphomal).
        in: Sommers S.C. Rosen P.P. Gastrointestinal and Hepatic Pathology Decennial 1965–1975. Appleton-Century-Crofts, New York1975: 131-160
        • Haghighi P.
        • Nasr K.
        Tropical sprue: Subclinical and idiopathic enteropathy.
        in: Sommers S.C. Rosen P.P. Pathology Annual. Appleton-Century-Crofts, East Norwalk, Connecticut1975: 103-129
        • Haghighi P.
        • Kharazmi A.
        • Gerami C.
        • et al.
        Primary upper small intestinal lymphoma and alpha-chain disease.
        Am. J. Surg. Pathol. 1978; 2: 147
        • Haghighi P.
        • Tabei Z.
        • Kharazmi A.
        • et al.
        Immunoperoxidase study in alpha-chain disease.
        Arch. Pathol. Lab. Med. 1978; 102: 555
        • Haghighi P.
        Primary small intestinal lymphoma and immunoproliferative small intestinal disease: An update.
        in: Sommers S.C. Rosen P.P. Malignant Lymphomas, a Pathology Annual Monograph. Appleton-Century-Crofts, East Norwalk, Connecticut1983: 269-295
        • Harris O.D.
        • Cooke W.T.
        • Thompson H.
        • Waterhouse J.A.H.
        Malignancy in adult celiac disease and idiopathic steatorrhea.
        Am. J. Med. 1967; 42: 899
        • Hermans P.E.
        • Diaz-Buyo J.A.
        • Stobo J.D.
        Idiopathic late-onset immunoglobulin deficiency: Clinical observations in 50 patients.
        Am. J. Med. 1976; 61: 221

      40. Isaacson, P., and Wright, D. H.: Malabsorption and intestinal lymphomas. In Wright, R. (ed.): Recent Advances in Gastrointestinal Pathology. Clin. Gastroenterol., Suppl. 1, 1980, pp. 193–212.

        • Isaacson P.
        Primary gastrointestinal lymphoma.
        Virchows Arch. (Pathol. Anat.). 1981; 391 (editorial): 1
        • Isaacson P.
        • Al-Dewachi H.S.
        • Mason D.Y.
        Middle Eastern intestinal lymphoma: A morphological immunohisto-chemical study.
        J. Clin. Pathol. 1983; 36: 489
        • Isaacson P.
        • Wright D.H.
        Malignant Lymphoma of mucosa-associated lymphoid tissue: A distinctive type of B-cell lymphoma.
        Cancer. 1983; 52: 1410
        • Isaacson P.G.
        • Maclenna K.A.
        • Subbuswamy S.G.
        Multiple lymphomatous polyposis of the gastrointestinal tract.
        Histopathology. 1984; 8: 641
        • Isaacson P.
        • Wright D.H.
        Extranodal malignant lymphoma arising from mucosa associated lymphoid tissue.
        Cancer. 1984; 53: 2515
        • Isaacson P.G.
        • Price S.K.
        Light chains in Mediterranean lymphoma.
        J. Clin. Pathol. 1985; 38: 601
        • Khojasteh A.
        • Haghshenass M.
        • Haghighi P.
        Current concepts: Immunoproliferative small intestine disease, a “Third World” lesion.
        N. Engl. J. Med. 1983; 308: 1401
        • Kopec M.
        • Swierczynska Z.
        • Pazdur J.
        • et al.
        Diffuse lymphoma of the intestine with a monoclonal gammopathy of IgG3 kappa type.
        Am. J. Med. 1974; 56: 381
        • Lewin K.J.
        • Kahn L.B.
        • Novis B.H.
        Primary intestinal lymphoma of “Western” and “Mediterranean” type, alpha-chain Disease and massive plasma all infiltration: A comparative study of 37 cases.
        Cancer. 1976; 8: 2511
        • Lewin K.J.
        • Ranchod M.
        • Dorfman R.F.
        Lymphomas of the gastrointestinal tract: A study of 117 cases presenting with gastrointestinal disease.
        Cancer. 1978; 42: 693
        • Manousos D.N.
        • Economidou J.C.
        Localized plasmacytoma in a patient with alpha-chain disease in remission.
        Br. Med. J. 1975; 4: 758

      52. Manousos, D. N., Economidou, J., Nicolaou, A., etal.: Spectrum of immunoproliferative small intestinal disease (IPSID) in Greece (abstract). 7th World Congress of Gastroenterology, June 1982, Stockholm, Sweden. Scand. J. Gastroenterol. Suppl. 78(to Vol. 17), Abstr. 1117, 1982, p. 281.

        • Alatuchanskv C.
        • Preud’homme J.L.
        • Babin P.
        Letter to the Editor.
        N. Engl. J. Med. 1983; 309: 1126
        • Matuchansky C.
        • Touchard G.
        • Lemaire M.
        • et al.
        Malignant lymphoma of the small bowel associated with diffuse nodular lymphoid hyperplasia.
        N. Engl. J. Med. 1985; 313: 166
        • Moore I.
        • Wright D.H.
        Primary gastric lymphoma—a tumor of mucosa-associated lymphoid tissue. A histological and immunohistochemical study of 36 cases.
        Histopathology. 1984; 8: 1025
        • Najem A.Z.
        • Porcaro J.L.
        • Rush B.F.
        Primary non-Hodgkin’s lymphoma of the duodenum.
        Cancer. 1984; 54: 895
        • Nasr K.
        • Haghighi P.
        • Abadi P.
        • et al.
        Idiopathic enteropathy: An evaluation in rural Iran with an appraisal of nutrient loss.
        Am. J. Clin. Nutr. 1976; 29: 169
        • Nassar V.H.
        • Salem P.A.
        • Shahid M.J.
        • et al.
        “Mediterranean” abdominal lymphoma or immunoproliferative small intestinal disease. Part II. Pathologic aspects.
        Cancer. 1978; 41: 1340
        • Nemes Z.
        • Thomazy V.
        • Szeifert G.
        Follicular center cell lymphoma with alpha-heavy chain disease. An histopathological and immunohistological study.
        Virchows Arch. (Pathol. Anat.). 1981; 394: 119
        • Neudorf S.
        • Snover D.
        • Filipovich A.
        Immunoproliferative small intestinal disease.
        N. Engl. J. Med. 1983; 309: 1126
        • Novis B.H.
        • King H.S.
        • Gilinsky N.H.
        • et al.
        Long survival in a patient with alpha-chain disease.
        Cancer. 1984; 53: 970
        • Omar Y.T.
        • Al-Nakib B.
        • Temmin S.M.A.L.
        • et al.
        Primary gastrointestinal lymphoma in Kuwait. An 11-year analysis of 108 cases.
        Eur. J. Cancer Clin. Oncol. 1985; 21: 573
        • Otto H.F.
        • Bettaman I.
        • Weltzien J.V.
        • et al.
        Primary intestinal lymphomas.
        Virchows Arch. (Pathol. Anat.). 1981; 391: 9
        • Preud’homme J.L.
        • Brouet J.C.
        • Seligmann M.
        Cellular immunoglobulins in human gamma and alpha-heavy chain diseases.
        Clin. Exp. Immunol. 1979; 37: 283
        • Rambaud J.C.
        • Bognel C.
        • Prost A.
        • et al.
        Clinicopathological study of a patient with “Mediterranean” type of abdominal lymphoma and a new type of IgA abnormality (alpha chain disease).
        Digestion. 1968; 1: 321
        • Rambaud J.C.
        • Galian A.
        • Danon F.G.
        • et al.
        Alpha-chain disease without quantitative serum IgA abnormality. Report of two cases, including a “non-secretory” form.
        Cancer. 1983; 51: 686
        • Ramot B.
        • Levanon M.
        • Hahn Y.
        • et al.
        The mutual clonal origin of the lymphoplasmacvtic and lvmphoma cell in alpha-heavy chain disease.
        Clin. Exp. Immunol. 1977; 27: 440
        • Ramot B.
        • Shahin N.
        • Bubis J.J.
        Malabsorption syndrome in lymphoma of small intestine. A study of 13 cases.
        Isr. J. Med. Sci. 1965; 1: 221
        • Rappaport H.
        The pathologic anatomy of alpha-chain disease.
        Hämatologie und Blut transfusion. 1976; 18: 337
        • Savilahti E.
        • Brandtzaeg P.
        An atypical Case of alpha-chain disease.
        Scand. J. Immunol. 1973; 2: 322
        • Savilahti E.
        • Brandtzaeg P.
        • Kuitunen P.
        A typical intestinal alpha-chain disease evolving into selective immunoglobulin A deficiency in a Finnish boy.
        Gastroenterologv. 1980; 79: 1303
        • Seligmann M.
        Alpha-chain disease.
        Gastroenterology. 1972; 63: 914
        • Seligmann M.
        Immunobiology and pathogenesis of alpha-chain disease.
        in: Ciba Found. Svmp, 46(new series). Elsevier Excerpta Med, North Holland1977: 263-281
        • Seligmann M.
        Les maladies des chaines lourdes.
        Rev. Prat. 1979; 29: 373
        • Seligmann M.
        • Danon F.
        • Hurez D.
        • et al.
        Alpha-chain disease: A new immunoglobulin abnormality.
        Science. 1968; 162: 1396
        • Seligmann M.
        • Danon F.
        • Preud’homme J.L.
        • et al.
        Aspects immunologiques de la maladie des chaines alpha.
        Acta Gastroenterol. Belg. 1978; XII: 264
        • Seligmann M.
        • Mihaesco E.
        • Preud’homme J.L.
        • et al.
        Heavy chain diseases: Current findings and concepts.
        Immunol. Rev. 1979; 43: 145
        • Seligmann M.
        • Rambaud J.C.
        Alpha-chain disease: An immunoproliferative disease of the secretory immune system.
        Ann. N.Y. Acad. Sci. 1983; 409: 478
        • Sherlock P.
        • Winawer S.
        • Goldstein M.
        • et al.
        Malignant lymphoma of the gastrointestinal tract.
        in: Jerzv-Glass G. Progress in Gastroenterology. Volume 2. Grune and Stratton, New York1970: 367-391
        • Skinner J.M.
        Gastrointestinal lymphomas.
        Pathology. 1985; 17: 193
        • Skrimshire J.F.P.
        Lymphoma of the stomach and intestine.
        Ģ. J. Med. 1955; 24: 203
        • Sleisenger M.H.
        • Almy T.P.
        • Barr D.P.
        The sprue syndrome secondary to lymphoma of the small bowel.
        Am. J. Med. 1953; 15: 666
        • Tangun Y.
        • Saracbasi A.
        • Incemen S.
        • et al.
        IgA myeloma globulin and Bence Jones proteinuria in plasmacytoma of small intestine.
        Ann. Intern. Med. 1975; 83: 673
        • Tucker P.W.
        • Slightom J.L.
        • Blattner F.
        Mouse IgA heavy chain gene sequence: Implications for evolution of immunoglobulin hinge exons.
        Proc. Natl. Acad. Sci. USA. 1981; 78: 7684
        • Ulmann A.
        • Abeshouse B.S.
        Lymphosarcoma of the small and large intestines.
        Ann. Surg. 1932; 95: 878
        • WHO memorandum
        Alpha-chain disease and related small intestinal lymphoma: A memorandum.
        Bull. WHO. 1976; 54: 615
        • 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. USA. 1974; 71: 974

      Article info

      Identification

      DOI: https://doi.org/10.1016/S0272-2712(18)30794-7

      Copyright

      © 1986 Elsevier Inc. Published by Elsevier Inc. All rights reserved.

      ScienceDirect

      Access this article on ScienceDirect

      The content on this site is intended for healthcare professionals.


      We use cookies to help provide and enhance our service and tailor content. To update your cookie settings, please visit the Cookie Preference Center for this site.
      Copyright © 2023 Elsevier Inc. except certain content provided by third parties.


      RELX