Owen R G, Lubenko A, Savage J, Parapia L A, Jack A S, Morgan G J
Department of Hematology, Bradford Royal Infirmary, United Kingdom.
Am J Hematol. 2001 Feb;66(2):116-9. doi: 10.1002/1096-8652(200102)66:2<116::AID-AJH1026>3.0.CO;2-V.
Autoimmune phenomena are well-recognised complications of Waldenström's macroglobulinemia (WM) and IgM monoclonal gammopathy. Peripheral neuropathy and cold agglutinin hemolytic anemia are the most common reported and occur in 5-10% of patients. Autoimmune thrombocytopenia has been rarely reported in WM and its incidence is not known. In this study we report the case of a 67-year-old man who presented with autoimmune thrombocytopenia who was subsequently found to have WM. Laboratory investigation demonstrated that platelet-associated IgM (PAIgM) but not PAIgG was clearly elevated compared to normal controls. In addition the patient's serum reacted strongly with a panel of donor platelets analysed with an indirect platelet immunofluorescence assay utilising an anti-IgM secondary antibody. Glycoprotein specificity could not however be demonstrated by ELISA techniques for platelet glycoproteins IIbIIIa, IaIIa, IbIXa, and IV. We also reviewed the case records of 104 additional cases of WM diagnosed at our institution between 5/93 and 5/99. Three further cases with clinically significant autoimmune thrombocytopenia were identified. The overall incidence of autoimmune thrombocytopenia (4/105, 3.8%) in this cohort of patients was similar to the incidence of peripheral neuropathy (7/105, 6.7%) and cold agglutinins (3/105, 2.9%).
自身免疫现象是华氏巨球蛋白血症（WM）和IgM单克隆丙种球蛋白病公认的并发症。周围神经病变和冷凝集素溶血性贫血是最常报道的，见于5% - 10%的患者。自身免疫性血小板减少症在WM中鲜有报道，其发病率未知。在本研究中，我们报告了一例67岁男性患者，其表现为自身免疫性血小板减少症，随后被发现患有WM。实验室检查表明，与正常对照相比，血小板相关IgM（PAIgM）而非PAIgG明显升高。此外，患者血清与一组供体血小板强烈反应，该反应通过使用抗IgM二抗的间接血小板免疫荧光测定法进行分析。然而，血小板糖蛋白IIbIIIa、IaIIa、IbIXa和IV的ELISA技术无法证明糖蛋白特异性。我们还回顾了1993年5月至1999年5月期间在我们机构诊断的另外104例WM病例记录。又发现了3例具有临床意义的自身免疫性血小板减少症病例。该组患者中自身免疫性血小板减少症的总体发病率（4/105，3.8%）与周围神经病变（7/105，6.7%）和冷凝集素（3/105，2.9%）的发病率相似。
