PMID: 6401593Feb 15, 1983Paper

Alpha-chain disease without qualitative serum IgA abnormality. Report of two cases, including a "nonsecretory" form

Cancer
J C RambaudA Chapman

Abstract

Two cases of alpha-chain disease (alpha-CD) without detectable amounts of alpha-CD protein in serum are reported. Both patients presented with the usual epidemiologic, clinical and pathologic features of alpha-CD, including a predominantly plasmacytic infiltration of the whole small intestine, alpha-CD Protein was found in the jejunal fluid in one case but not in the other, the latter presenting as a "nonsecretory" form of alpha-CD. In both cases, immunofluorescence study of the small bowel mucosa showed that most of the infiltrating cells were positive for alpha chains and negative for other isotypes, and kappa and lambda light chains. These findings warrant a reevaluation of the prevalence of alpha-CD protein synthesis in immunoproliferative small intestinal disease (IPSID) (including lymphomas previously described as Mediterranean lymphoma) by performing an adequate search for alpha-CD protein in the jejunal juice, and at the cellular level in patients without detectable amounts of this protein in serum.

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Citations

Jan 8, 2000·Hematology/oncology Clinics of North America·J P Fermand, J C Brouet
Jan 26, 1999·Journal of Gastroenterology and Hepatology·S NairV I Mathan
Jun 1, 1986·Gut·J CoulboisJ Rambaud
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Jan 1, 1988·Critical Reviews in Oncology/hematology·R A Kyle, P A Greipp
Jul 20, 2005·Best Practice & Research. Clinical Haematology·Dietlind L Wahner-Roedler, Robert A Kyle
May 18, 2001·Best Practice & Research. Clinical Gastroenterology·A Ruskoné-Fourmestraux, J C Rambaud
Jan 1, 1994·The British Journal of Surgery·I G Martin, M I Aldoori
Jun 8, 1989·The New England Journal of Medicine·C MatuchanskyJ L Preud'homme
Aug 9, 2006·European Journal of Gastroenterology & Hepatology·Ioanna EconomidouTheodora Papadakis
Apr 1, 1985·Pathology·J M Skinner

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