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Alpha‐chain disease without qualitative serum IgA abnormality: Report of two cases, including a “nonsecretory” form
Author(s) -
Rambaud JeanClaude,
Galian Annie,
Da Francloise G.,
Preud'Homme JeanLouis,
Brandtzaeg Per,
Wassef Michel,
Carrer Michel Le,
Mehaut Marcel A.,
Voinchet Odile L.,
Perol Roland G.,
Chapman Antoine
Publication year - 1983
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19830215)51:4<686::aid-cncr2820510423>3.0.co;2-#
Subject(s) - alpha chain , alpha (finance) , medicine , lymphoma , pathology , antibody , immunoglobulin a , immunofluorescence , immunoglobulin light chain , disease , gastroenterology , kappa , infiltration (hvac) , immunology , immunoglobulin g , linguistics , philosophy , receptor , nursing , physics , patient satisfaction , thermodynamics , construct validity
Two cases of α‐chain disease (α ‐CD) without detectable amounts of α ‐CD protein in serum are reported. Both patients presented with the usual epidemiologic, clinical and pathologic features of α ‐CD, including a predominantly plasmacytic infiltration of the whole small intestine. α ‐CD Protein was found in the jejunal fluid in one case but not in the other, the latter presenting as a “nonsecretory” form of α ‐CD. In both cases, immunofluorescence study of the small bowel mucosa showed that most of the infiltrating cells were positive for α chains and negative for other isotypes, and Λ light chains. These findings warrant a reevaluation of the prevalence of α‐CD protein synthesis in immunoproliferative small intestinal disease (IPSID) (including lymphomas previously described as Mediterranean lymphoma) by performing an adequate search for α‐CD protein in the jejunal juice, and at the cellular level in patients without detectable amounts of this protein in serum.