title: Anti-Sa cold agglutinin of IgA class requiring plasma-exchange therapy as early manifestation of multiple myeloma.
abstract: The case of a patient (Col) with multiple myeloma presenting as chronic cold agglutinin (CA) syndrome is reported. The CA (Col) was a monoclonal IgA/k paraprotein which recognizes an antigen fully expressed in adult and newborn erythrocytes, sialidase sensitive and partially resistant to proteases. Hemagglutination-inhibition studies showed that immunodominant N-acetylneuraminic acid bound alpha 2-->3 to O-glycans of glycophorins represents the CA(Col) epitope. These serological and biochemical findings fit with the anti-Sa specificity, of which only two previous examples are known. The clinical manifestations of CA (Col) were characterized by marked acrocyanosis, generalized livedo reticularis, and incapacitating dyspnea, but only mild hemolysis. Plasma-exchange therapy was effective in quickly removing the CA and relieving the associated clinical manifestations, but such benefit was only temporary. This is the first reported example of anti-Sa CA of IgA isotype and the first case of IgA CA syndrome treated by plasma exchange.
authors.affiliation: Service of Hemotherapy and Hemostasis, Hospital Clínico, Barcelona, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Pereira
authors.forename: R
authors.initials: R
authors.lastname: Mazzara
authors.forename: L
authors.initials: L
authors.lastname: Escoda
authors.forename: I
authors.initials: I
authors.lastname: Alcorta
authors.forename: B
authors.initials: B
authors.lastname: Nomdedeu
authors.forename: D
authors.initials: D
authors.lastname: Roelcke
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis* / Q000601:surgery; D003937:Diagnosis, Differential; D006385:Hemagglutination Inhibition Tests; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D007132:Immunoglobulin Isotypes / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000175:diagnosis*; D010951:Plasma Exchange
chemical_list: D007070:Immunoglobulin A; D007132:Immunoglobulin Isotypes