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著者: Annette H M Van Der Helm-Van Mil, Alice C Smith, Shideh Pouria, Edward Tarelli, Nigel J Brunskill, Heroen C J Eikenboom
雑誌名: Br J Haematol. 2003 Sep;122(6):915-7.
Abstract/Text
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.
PMID 12956761 Br J Haematol. 2003 Sep;122(6):915-7.
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