Artigo Revisado por pares

Severe Diarrhea Controlled by Gamma Globulin in a Patient with Agammaglobulinemia, Amyloidosis, and Thymoma

1966; American College of Physicians; Volume: 65; Issue: 3 Linguagem: Inglês

10.7326/0003-4819-65-3-528

ISSN

1539-3704

Autores

Harold O. Conn,

Tópico(s)

Pancreatitis Pathology and Treatment

Resumo

Case Studies1 September 1966Severe Diarrhea Controlled by Gamma Globulin in a Patient with Agammaglobulinemia, Amyloidosis, and ThymomaHAROLD O. CONN, M.D., RICHARD QUINTILIANI, M.D.HAROLD O. CONN, M.D.Search for more papers by this author, RICHARD QUINTILIANI, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-65-3-528 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe description of agammaglobulinemia by Bruton (1) in 1952 initiated reports of a variety of hypoagammaglobulinemic syndromes and stimulated an enormous amount of research in infectious disease and immunology. Among the clinical variants of hypogammaglobulinemia reported were sprue-like syndromes, anemias of diverse type, and a high prevalence of thymoma. The present report concerns a patient with adult-onset, "acquired" hypogammaglobulinemia who had severe diarrhea which was controlled by the administration of gamma globulin. In addition, amyloidosis and a thymoma were found at autopsy. The purpose of this paper is to define possible relationships among these abnormalities by reviewing the previously reported...References1. BRUTON OC: Agammaglobulinemia. Pediatrics 9: 722, 1952. MedlineGoogle Scholar2. GITLIN D: The hypogammaglobulinemias. DM May, 1962, p. 1. Google Scholar3. CERFCOHEN-SOLALCATTAN MJR: Les hypogammaglobulinémies de l'adulte Presumées Idiopathiques. Le Sang 29: 177, 1958. MedlineGoogle Scholar4. SANFORDFAVOURTRIBEMAN JPCBMS: Absence of serum gamma globulins in an adult. New Eng. J. Med. 250: 1027, 1954. CrossrefMedlineGoogle Scholar5. ROHNBEHNKEBOND RJRHWH: Acquired hypogammaglobulinemia with hypersplenism. A case report. Amer. J. Med. Sci. 229: 406, 1955. CrossrefMedlineGoogle Scholar6. ROSECANTROBAUGHDANFORTH MFEWH: Agammaglobulinemia in the adult. Amer. J. Med. 19: 303, 1955. CrossrefMedlineGoogle Scholar7. WALLSASLAW RLS: Adult agammaglobulinemia. Arch. Intern. Med. (Chicago) 95: 33, 1955. CrossrefGoogle Scholar8. BARUNDUNBÜCHLERHASSIG SHA: Das Antikorpermangelsyndrom. Agammaglobulinämie. Schweiz. Med. Wschr. 86: 33, 1956. MedlineGoogle Scholar9. RAMOS AJ: Agammaglobulinemia and thymoma. Presentation of a case. JAMA 160: 1317, 1956. CrossrefMedlineGoogle Scholar10. LEWISBROWN ECHE: Agammaglobulinemia associated with pernicious anemia and diabetes mellitus. Arch. Intern. Med. (Chicago) 100: 296, 1957. CrossrefGoogle Scholar11. SANDORKAZMER TJ: Agammaglobulinemia Whipple Betesegre Emlekezteto Syndromaban. Magy. Belorv. Arch. 10: 97, 1957. Google Scholar12. ZELMANLEWIN SH: Adult agammaglobulinemia associated with multiple congenital abnormalities. Amer. J. Med. 25: 150, 1958. CrossrefMedlineGoogle Scholar13. BERNHEIMCREYSSELGILLYFOURNIER MRRP: Agammaglobulinémie congenitale avec stéatorrhée chez deux jumeaux hétérozygotes de sexe different. Pediatrie 14: 881, 1959. MedlineGoogle Scholar14. MALVEZINCONTELAGENTEJAULIN JMBP: Sprue chez un agammaglobulinémique. Arch. Mal. App. Dig. 48: 504, 1959. MedlineGoogle Scholar15. KABLER JD: Rare malabsorption syndromes. Ann. Intern. Med. 52: 1221, 1960. LinkGoogle Scholar16. COHENCOHENCOHEN JALD: Agammaglobulinemia con Esteatorrhoea y Probable Tuberculosis. Presna Med. Argent. 48: 2203, 1961. MedlineGoogle Scholar17. COHENPALEYJANOWITZ NDHD: Acquired hypogammaglobulinemia and sprue: report of a case and review of the literature. J. Mt. Sinai Hosp. 28: 421, 1961. MedlineGoogle Scholar18. GARVIEKENDALL JMAC: Congenital agammaglobulinemia. Report of 2 further cases. Brit. Med. J. 1: 548, 1961. CrossrefMedlineGoogle Scholar19. HUIZENGAWOLLAEGERGREENMCKENZIE KAEEPABF: Serum globulin deficiencies in non-tropical sprue, with report of two cases of acquired agammaglobulinemia. Amer. J. Med. 31: 572, 1961. CrossrefMedlineGoogle Scholar20. GREENSPERBER IRJ: Hypogammaglobulinemia, arthritis, sprue, and megaloblastic anemia. New York J. Med. 62: 1679, 1962. MedlineGoogle Scholar21. HOLLINGWORTH GT: Congenital hypogammaglobulinaemia and megaloblastic anaemia presenting as a case of bronchiectasis. Proc. Roy. Soc. Med. 55: 606, 1962. CrossrefGoogle Scholar22. SWIFT PN: Hypogammaglobulinemia, steatorrhea and megaloblastic anemia. Response to gluten-free diet and folic acid. Postgrad. Med. J. 38: 633, 1962. CrossrefMedlineGoogle Scholar23. PELKONENSIURALAVUOPIO RMP: Inherited agammaglobulinemia with malabsorption and marked alterations in the gastrointestinal mucosa. Acta Med. Scand. 173: 549, 1963. CrossrefGoogle Scholar24. Case records of the Massachusetts General Hospital (Case 6, 1963). New Eng. J. Med. 268: 204, 1963. CrossrefGoogle Scholar25. ALLENHADDEN GEDR: Congenital hypogammaglobulinaemia with steatorrhea in two adult brothers. Brit. Med. J. 2: 486, 1964. CrossrefMedlineGoogle Scholar26. WALDMANNLASTER TAL: Abnormalities of albumin metabolism in patients with hypogammaglobulinemia. J. Clin. Invest. 43: 1025, 1962. CrossrefGoogle Scholar27. WOLLHEIMCOSTERLINDHOLM FABCH: Primary "acquired" hypogammaglobulinemia. Acta Med. Scand. (suppl.) 176: 415, 1964. Google Scholar28. COLLINSELLIS JRDS: Agammaglobulinemia, malabsorption and rheumatoid arthritis. Amer. J. Med. 39: 476, 1965. CrossrefMedlineGoogle Scholar29. FORSSMANHERNER OB: Acquired agammaglobulinaemia and malabsorption. Acta Med. Scand. 176: 779, 1965. CrossrefGoogle Scholar30. MCCARTHYAUSTADREAD CFWIAE: Hypogammaglobulinemia and steatorrhea. Amer. J. Dig. Dis. 10: 945, 1965. CrossrefMedlineGoogle Scholar31. ZINNEMANHALL HHWH: Steatorrhea and probable tuberculosis with acquired agammaglobulinemia. Amer. Rev. Tuberc. Pul. Dis. 74: 773, 1956. Google Scholar32. COOKEWEINERSHINTON WTWNK: Agammaglobulinemia. Report of two adult cases. Brit. Med. J. 1: 1151, 1957. CrossrefMedlineGoogle Scholar33. FIRKINBLACKBURN BCCR: Congenital and acquired agammaglobulinaemia. Quart. J. Med. 27: 187, 1958. MedlineGoogle Scholar34. HOLMANNICKELSLEISENGER HWFMH: Hypoproteinemia antedating intestinal lesions, and possibly due to excessive serum protein loss into the intestine. Amer. J. Med. 27: 963, 1959. CrossrefMedlineGoogle Scholar35. FRENCH JM: Problems raised by the treatment of steatorrhoea with antibacterial drugs. Postgrad. Med. J. 37: 259, 1961. CrossrefMedlineGoogle Scholar36. DONALDSON RM: Editorial: Malabsorption in the blind loop syndrome. Gastroenterology 48: 388, 1965. CrossrefGoogle Scholar37. COLLINS JR: Small intestinal mucosal damage with villous atrophy. Amer. J. Clin. Path. 44: 36, 1965. CrossrefMedlineGoogle Scholar38. KINGJOSKE MJRA: Acute enteritis with temporary intestinal malabsorption. Brit. Med. J. 1: 1324, 1960. CrossrefMedlineGoogle Scholar39. SPRINZ H: Morphologic response of intestinal mucosa to enteric bacteria and its implication for sprue and Asiatic cholera. Fed. Proc. 21: 57, 1962. MedlineGoogle Scholar40. ABRAMSBAUERSPRINZ GDHH: Influence of normal flora on mucosal morphology and cellular renewal in the ileum. A comparison of germ-free and conventional mice. Lab. Invest. 12: 355, 1963. MedlineGoogle Scholar41. TRIERPHELPSEIDELMANRUBIN JSPCSCE: Whipple's disease: light and electron microscope correlation of jejunal mucosal histology with antibiotic treatment and clinical status. Gastroenterology 48: 684, 1965. CrossrefMedlineGoogle Scholar42. ARNOLD L: Alterations in the endogenous enteric bacterial flora and microbic permeability of the intestinal wall in relation to the nutritional and meteorological changes. J. Hyg. 29: 82, 1929. CrossrefMedlineGoogle Scholar43. REITHSQUIER AFTL: Blood cultures of apparently healthy persons. J. Infect. Dis. 51: 336, 1932. CrossrefGoogle Scholar44. SCHWEINBURGSELIGMANFINE FBAMJ: Transmural migration of intestinal bacteria. A study based on the use of radioactive Escherischia coli. New Eng. J. Med. 242: 747, 1950. CrossrefMedlineGoogle Scholar45. CONN HO: Spontaneous peritonitis and bacteremia in Laennec's cirrhosis caused by enteric organisms. A relatively common but rarely recognized syndrome. Ann. Intern. Med. 60: 568, 1964. LinkGoogle Scholar46. FREUNDLICH E: Agammaglobulinemia. J. Pediatrics 50: 475, 1957. CrossrefMedlineGoogle Scholar47. MARTINGORDONMCCULLOCH CMRSNB: Acquired hypogammaglobulinemia in an adult. Report of a case with clinical and experimental studies. New Eng. J. Med. 254: 449, 1956. CrossrefMedlineGoogle Scholar48. FRENCHHALLPARISHSMITH JMGDJWT: Peripheral and autonomic nerve involvement in primary amyloidosis associated with uncontrollable diarrhoea and steatorrhoea. Amer. J. Med. 39: 277, 1965. CrossrefMedlineGoogle Scholar49. SCHEIN J: Syndrome of nontropical sprue with hitherto undescribed lesions of the intestine. Gastroenterology 8: 438, 1947. MedlineGoogle Scholar50. DAHLIN DC: Secondary amyloidosis. Ann. Intern. Med. 31: 105, 1949. LinkGoogle Scholar51. BERO GL: Amyloidosis: its clinical and pathologic manifestations with a report of 12 cases. Ann. Intern. Med. 46: 931, 1957. LinkGoogle Scholar52. BRIGGS GW: Amyloidosis. Ann. Intern. Med. 55: 943, 1961. LinkGoogle Scholar53. GREENHIGGINSBROWNHOFFMANSOMMERVILLE PAJAALHNRL: Amyloidosis: appraisal of intubation biopsy of the small intestine in diagnosis. Gastroenterology 41: 452, 1961. CrossrefMedlineGoogle Scholar54. GRASLATORREGAMISSANS JJJM: Hypoagamma-globulinämie bei einem fall von Amyloidose. Klin. Wschr. 32: 968, 1954. CrossrefMedlineGoogle Scholar55. Clinicopathological Conference. A case of primary acquired hypogammaglobulinaemia with pernicious anaemia and amyloidosis. Brit. Med. J. 1: 35, 1965. CrossrefMedlineGoogle Scholar56. TEILUM G: Periodic acid-Schiff positive reticuloendothelial cells producing glycoprotein. Functional significance during formation of amyloid. Amer. J. Path. 32: 945, 1956. MedlineGoogle Scholar57. VASQUEZDIXON JJFJ: Immunohistochemical analysis of amyloid by the fluorescent technique. J. Exp. Med. 104: 727, 1956. CrossrefMedlineGoogle Scholar58. OSSERMAN EF: Amyloidosis: tissue proteinosis: gammaloidosis. Ann. Intern. Med. 55: 1033, 1961. LinkGoogle Scholar59. OSSERMANTAKATSUKITALAL EFKN: Multiple myeloma I. The pathogenesis of "amyloidosis." Seminar Hemat. 1:3, 1964. MedlineGoogle Scholar60. MACLEANZAKVARCOGOOD LDSJRLRA: Thymic tumor and acquired agammaglobulinemia: a clinical and experimental study of the immune response. Surgery 40: 1010, 1956. MedlineGoogle Scholar61. LAMBIEBURROWSSOMMERS ATBASC: Clinicopathologic conference. Refractory anemia agammaglobulinemia and mediastinal tumor. Amer. J. Clin. Path. 27: 444, 1957. CrossrefMedlineGoogle Scholar62. GAFNIMICHAELIHELLER JDH: Idiopathic acquired agammaglobulinemia associated with thymoma. Report of two cases and review of the literature. New Eng. J. Med. 263: 536, 1960. CrossrefMedlineGoogle Scholar63. GODFREY S: Thymoma with hypogammaglobulinemia in an identical twin. Brit. Med. J. 1: 1159, 1964. CrossrefMedlineGoogle Scholar64. JACOXMONGANHANSHAWLEDDY RFESJBJP: Hypogammaglobulinemia with thymoma and probable pulmonary infection with cytomegalovirus. New Eng. J. Med. 271: 1091, 1964. CrossrefMedlineGoogle Scholar65. CHAPIN MA: Benign thymoma, refractory anemia and hypogammaglobulinemia. J. Maine Med. Ass. 56: 83, 1965. MedlineGoogle Scholar66. GEHRMANNENGSTFELD GG: Thymoma and agammaglobulinaemia. German Med. Monthly 10: 281, 1965. MedlineGoogle Scholar67. GOODGABRIELSON RAAE: The Thymus in Immunobiology. Structure, Function, and Role in Disease. Hoeber Medical Division, Harper & Row, New York, 1964. Google Scholar68. HERMANSHUIZENGAHOFFMANBROWNMARKOWITZ PEKAHNALH: Dysgammaglobulinemia associated with nodular lymphoid hyperplasia of the small intestine. Amer. J. Med. 78: 89, 1966. Google Scholar69. CRABBEHEREMANS PAJF: Lack of gamma A-immunoglobulin in serum of patients with steatorrhoea. Gut 7: 199, 1966. CrossrefGoogle Scholar70. EIDELMANDAVISLAGUNOFFRUBIN SSDDCE: The relationship between intestinal plasma cells and serum immunoglobulin A (IgA) in man. J. Clin. Invest. 45: 1003, 1966. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: West Haven, Newington, and New Haven, ConnecticutFrom the Veterans Administration Hospitals, West Haven and Newington, and the Department of Internal Medicine, New Haven, Conn.This study was supported by a grant from the Stratfield Fund.Requests for reprints should be addressed to H. O. Conn, M.D., Veterans Administration Hospital, West Haven, Conn. 06516. 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