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Role of light chain variable region in myeloma with light chain deposition disease: evidence from an experimental model

1995; Elsevier BV; Volume: 86; Issue: 10 Linguagem: Inglês

10.1182/blood.v86.10.3655.bloodjournal86103655

1528-0020

Ahmed Amine Khamlichi, Anna Rocca, Guy Touchard, Pièrre Aucouturier, JL Preud'homme, Michel Cogné,

Amyloidosis: Diagnosis, Treatment, Outcomes

Light chain deposition disease (LCDD) results from a propensity of some human monoclonal L chains to form tissue deposits. We designed an experimental model for in vivo expression of human kappa L chain sequences in mice and compared a somatically mutated LCDD chain with a closely related control kappa chain, both encoded by the unique V kappa IV gene. Mice secreting the LCDD chain but not those producing the control chain showed deposits with a distribution similar to that observed in patients. These data show that discrete changes in V region sequences can play a major role in tissue deposition of human L chains.