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华氏巨球蛋白血症:高黏滞综合征与冷球蛋白血症。

Waldenström's macroglobulinemia: hyperviscosity syndrome and cryoglobulinemia.

作者信息

Stone Marvin J

机构信息

Baylor Charles A. Sammons Cancer Center, Baylor University Medical Center, Dallas, TX 75246, USA.

出版信息

Clin Lymphoma Myeloma. 2009 Mar;9(1):97-9. doi: 10.3816/CLM.2009.n.026.

Abstract

Hyperviscosity syndrome (HVS) is a common manifestation of Waldenström's macroglobulinemia (WM). Patients with HVS have skin and mucosal bleeding, retinopathy with visual disturbances, and a variety of neurologic disorders. HVS can be diagnosed from physical examination by identifying the characteristic retinal venous engorgement ("sausaging") on funduscopic inspection. HVS can be accurately monitored with an Ostwald tube and reversed by plasmapheresis. Cryoglobulins precipitate or gel at temperatures < 37 degrees C and dissolve on re-warming. They may be composed of single or multiple components. Most cryoglobulins are mixed monoclonal IgMpolyclonal IgG immune complexes and many are associated with hepatitis C viral infection. Monoclonal macroglobulin autoreactive antibodies are included among the "IgM-related" disorders that influence the clinical presentation and natural history of WM.

摘要

高黏滞综合征(HVS)是华氏巨球蛋白血症(WM)的常见表现。HVS患者有皮肤和黏膜出血、伴有视力障碍的视网膜病变以及多种神经疾病。通过眼底检查发现特征性的视网膜静脉充血(“香肠样”),可从体格检查中诊断HVS。HVS可用奥氏管准确监测,并通过血浆置换逆转。冷球蛋白在温度<37摄氏度时沉淀或形成凝胶,复温后溶解。它们可能由单一或多种成分组成。大多数冷球蛋白是混合性单克隆IgM-多克隆IgG免疫复合物,许多与丙型肝炎病毒感染有关。单克隆巨球蛋白自身反应性抗体包括在影响WM临床表现和自然病程的“IgM相关”疾病中。

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