巨球蛋白血症性视网膜病变一例

题：巨球蛋白血症致高黏滞综合征相关视网膜病变一例（英文题：Hyperviscosity-RelatedRetinopathyinWaldenstr?m’sMacroglobulinemia；作者：WoojinKim，EuiyongKweon；作者单位：医院）男性患者，60岁，双眼视物模糊2个月，单眼最佳矫正视力为10/20，眼底检查发现双眼视网膜静脉迂曲扩张，并有出血（图A和B）。荧光血管照影示视网膜静脉串珠样改变及多发性动脉瘤样病变（图C和D）。血清蛋白免疫固定法检测发现血清内IgM和k轻链异常增多。骨髓活检发现表达CD和CD20的血浆淋巴细胞浸润达（10%-13%）。通过对其进行7次血浆置换疗法，视力恢复到20/20，对其进行随访眼底检查后发现该患者的双眼视网膜静脉异常也有明显好转。巨球蛋白血症是一种B淋巴细胞恶性增生性疾病，主要以骨髓中有浆细胞样淋巴细胞浸润并合成单克隆IgM为临床病理特征。此外，该病还会出现高黏滞综合征，可以表现为视力下降、皮肤出血以及其他神经症状，但很少出现该病例所出现的血管异常症状。A60-year-oldmanpresentedwitha2-monthhistoryofbilateralblurryvision.Thebestcorrectedvisualacuitywas10/20ineacheye.Funduscopicexaminationrevealedbilateralretinal-veindilatationandtortuositywithretinalhemorrhages(PanelsAandBshowtherightandlefteyes,respectively).Fluoresceinangiographyrevealedretinal-veinbeadingpatternswithmultipleaneurysmalchangesandvessel-wallstaining(PanelsCandD).SerumproteinimmunofixationassayshowedprominentmonoclonalbandsagainsttheantiserumofIgMandkappalightchains.Bonemarrowbiopsyrevealedinfiltrationofplasmacytoidlymphocytes(10to13%)withexpressionofCDandCD20markers.Aftersevenroundsofplasmapheresis,visualacuitygraduallyimprovedto20/20.Follow-upfunduscopicexaminationrevealedresolutionofretinal-veinabnormality.Waldenstr?m’smacroglobulinemiaisalymphoproliferativeB-celldisordercharacterizedbytheoverproductionofmonoclonalIgM.Personswiththisdisordercanpresentwiththehyperviscositysyndrome,whichcanmanifestasvisionchanges,cutaneousbleeding,andneurologicsymptoms.Vascularabnormalitiesarenotun







































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