Brouet Jean-Claude
Unité d'immuno-hématologie, Hôpital Saint-Louis, Paris.
Rev Prat. 2006 Jan 15;56(1):25-30.
Waldenström's macroglobulinemia associates a serum monoclonal IgM and a lymphoplasmacytic infiltration of bone marrow, spleen, lymph nodes and various organs. Clinical symptoms are related either to the lymphoid disorder or to the physico-chemical characteristics or antibody activity of the monoclonal IgM. Visceral infiltration may target stomach small bowel, lungs, exocrine glands or skin. Major complications include bone marrow failure, auto-immune cytopenia, occurrence of large cell lymphoma and infections (related either to the humoral immunodeficiency or to chemotherapy). Asymptomatic macroglobulinemia does not deserve any treatment. Otherwise, alkylating agents or nucleoside analogues are first line agents. The benefit of monoclonal antibodies, high dose chemotherapy followed by auto or allogenic stem cell graft is currently assessed.
华氏巨球蛋白血症伴有血清单克隆IgM以及骨髓、脾脏、淋巴结和各种器官的淋巴浆细胞浸润。临床症状要么与淋巴系统疾病有关,要么与单克隆IgM的物理化学特性或抗体活性有关。内脏浸润可能累及胃、小肠、肺、外分泌腺或皮肤。主要并发症包括骨髓衰竭、自身免疫性血细胞减少、大细胞淋巴瘤的发生以及感染(与体液免疫缺陷或化疗有关)。无症状性巨球蛋白血症无需任何治疗。否则,烷化剂或核苷类似物是一线治疗药物。目前正在评估单克隆抗体、大剂量化疗后自体或异基因干细胞移植的益处。