Immunoglobulin A multiple myeloma presenting with Henoch-Schönlein purpura associated with reduced sialylation of IgA1.
Henoch-Schönlein purpura is characterized by immunoglobulin A1 (IgA1) depositions in blood vessels of the skin or in glomeruli, resulting from altered hinge region O-glycosylation. Henoch-Schönlein purpura is seldom reported as a complication of IgA1 myeloma, even when the circulating IgA concentration is very high. We report two patients with IgA1 myeloma presenting with Henoch-Schönlein purpura. The O-glycosylation of these patients' IgA1 was studied. Both patients showed increased binding to peanut agglutinin lectin, suggesting a low degree of sialylation of the hinge region of IgA1 that was confirmed by mass spectrometry. IgA multiple myeloma, secreting IgA1 molecules with decreased sialylation, presenting with a Henoch-Schönlein purpura-like syndrome was diagnosed.
Van Der Helm-Van Mil
Annette H M
AH
Department of General Internal Medicine, Leiden University Medical Center, Leiden, The Netherlands. AvdHelm@lumc.nl
Smith
Alice C
AC
Pouria
Shideh
S
Tarelli
Edward
E
Brunskill
Nigel J
NJ
Eikenboom
Heroen C J
HC
D006031:Glycosylation; D006801:Humans; D011695:IgA Vasculitis / Q000097:blood / Q000209:etiology*; D007070:Immunoglobulin A / Q000097:blood*; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000097:blood / Q000150:complications*
D007070:Immunoglobulin A