Agammaglobulinemia, Plasma Cell Dyscrasia, and Amyloidosis in a 12-Year-Old Child

Albert I. Pick, Ishak Versano, Sara Schreibman, Mina Ben Bassat, Yehuda Shoenfeld

Research output: Contribution to journalArticlepeer-review

Abstract

A 12-year-old boy suffered from recurrent respiratory infections, an immune deficiency characterized by the complete absence of IgG, IgA, IgD, and the salivary “secretory component” associated with a plasma cell dyscrasia. Rectal and kidney biopsy specimens showed amyloid deposits. Amyloidosis has been extensively studied by several groups of investigators in patients with plasma cell dyscrasia in general, and in patients with λ-Bence Jones proteinuria in particular, but the finding of a monoclonal serum IgM-λ component in an agammaglobulinemic child with Bence Jones proteinuria and amyloidosis represents a puzzling clinical syndrome. Searching for monoclonal components in patients with amyloidosis, even if they are agammaglobulinemic, is important. We also discuss here the “mixed type” of amyloid deposition and its relation to the recurrent infections and the plasma cell dyscrasia in this patient.

Original languageEnglish
Pages (from-to)682-686
Number of pages5
JournalAmerican Journal of Diseases of Children
Volume131
Issue number6
DOIs
StatePublished - Jun 1977

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