Owen Roger G, Treon Steven P, Al-Katib Ayad, Fonseca Rafael, Greipp Philip R, McMaster Mary L, Morra Enrica, Pangalis Gerassimos A, San Miguel Jesus F, Branagan Andrew R, Dimopoulos Meletios A
Leeds General Infirmary, Leeds, UK.
Semin Oncol. 2003 Apr;30(2):110-5. doi: 10.1053/sonc.2003.50082.
This presentation represents consensus recommendations for the clinicopathological definition of Waldenstrom's macroglobulinemia (WM), which were prepared in conjunction with the Second International Workshop held in Athens, Greece during September 2002. WM is an uncommon lymphoproliferative disorder characterized primarily by bone marrow infiltration and IgM monoclonal gammopathy. It should be considered a distinct clinicopathological entity rather than a clinical syndrome secondary to IgM secretion. The underlying pathological diagnosis in WM is lymphoplasmacytic lymphoma as defined by the World Health Organization (WHO) and Revised European-American Lymphoma (REAL) classification criteria. The concentration of monoclonal IgM can vary widely in WM and it is not possible to define a concentration that reliably distinguishes WM from monoclonal gammopathy of undetermined significance (MGUS) and other lymphoproliferative disorders. A diagnosis of WM can therefore be made irrespective of IgM concentration if there is evidence on a bone marrow trephine biopsy of bone marrow infiltration by lymphoplasmacytic lymphoma with predominantly an intertrabecular pattern, supported by appropriate immunophenotypic studies. Simple criteria to distinguish patients with symptomatic WM who require therapy from those with asymptomatic WM and MGUS were also proposed. Patients with clinical features attributable to IgM monoclonal gammopathy but no overt evidence of lymphoma are considered to constitute a distinct clinical group and the term "IgM-related disorders" is proposed.
本报告代表了关于华氏巨球蛋白血症（WM）临床病理定义的共识性建议，这些建议是与2002年9月在希腊雅典举行的第二届国际研讨会共同制定的。WM是一种罕见的淋巴增殖性疾病，主要特征为骨髓浸润和IgM单克隆丙种球蛋白病。它应被视为一种独特的临床病理实体，而非继发于IgM分泌的临床综合征。WM的潜在病理诊断是世界卫生组织（WHO）和修订的欧美淋巴瘤（REAL）分类标准所定义的淋巴浆细胞淋巴瘤。在WM中，单克隆IgM的浓度差异很大，无法确定一个能可靠区分WM与意义未明的单克隆丙种球蛋白病（MGUS）及其他淋巴增殖性疾病的浓度。因此，如果骨髓穿刺活检有证据表明存在以小梁间模式为主的淋巴浆细胞淋巴瘤浸润，且有适当的免疫表型研究支持，那么无论IgM浓度如何，均可做出WM的诊断。还提出了区分需要治疗的有症状WM患者与无症状WM和MGUS患者的简单标准。具有归因于IgM单克隆丙种球蛋白病的临床特征但无明显淋巴瘤证据的患者被视为构成一个独特临床组，并提出了“IgM相关疾病”这一术语。
