Pereira A, Mazzara R, Escoda L, Alcorta I, Nomdedeu B, Roelcke D
Service of Hemotherapy and Hemostasis, Hospital Clínico, Barcelona, Spain.
Ann Hematol. 1993 Jun;66(6):315-8. doi: 10.1007/BF01695974.
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.
报告了一例多发性骨髓瘤患者（Col）表现为慢性冷凝集素（CA）综合征的病例。CA（Col）是一种单克隆IgA/k副蛋白，可识别在成人和新生儿红细胞中完全表达的抗原，对唾液酸酶敏感，对蛋白酶部分耐受。血凝抑制研究表明，免疫显性的N-乙酰神经氨酸以α2→3连接到血型糖蛋白的O-聚糖上代表了CA（Col）表位。这些血清学和生化结果符合抗-Sa特异性，此前仅已知两个例子。CA（Col）的临床表现以明显的肢端发绀、全身性网状青斑和使人衰弱的呼吸困难为特征，但仅有轻度溶血。血浆置换疗法可有效快速清除CA并缓解相关临床表现，但这种益处只是暂时的。这是首例报道的IgA同种型抗-Sa CA病例，也是首例通过血浆置换治疗的IgA CA综合征病例。
