Anti-Sa cold agglutinin of IgA class requiring plasma-exchange therapy as early manifestation of multiple myeloma.
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.
Pereira
A
A
Service of Hemotherapy and Hemostasis, Hospital Clínico, Barcelona, Spain.
Mazzara
R
R
Escoda
L
L
Alcorta
I
I
Nomdedeu
B
B
Roelcke
D
D
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
D007070:Immunoglobulin A; D007132:Immunoglobulin Isotypes