Waldenstrom's macroglobulinemia: clinical course and prognostic factors in 60 patients - Experience from a single hematology unit
Waldenstrom's macroglobulinemia: clinical course and prognostic factors in 60 patients - Experience from a single hematology unit
复制标题
DOI：
10.1007/s00277-001-0385-8
复制
发表时间：
2001-12-01
影响因子：
3.5
通讯作者：
Pangalis, GA
中科院分区：
文献类型：
--
作者：
Kyrtsonis, MC;Vassilakopoulos, TP;Pangalis, GA
瓦尔登斯特伦巨球蛋白血症（WM）是一种淋巴浆细胞性淋巴瘤，其特征是患者血清中存在I-M单克隆成分。我们报告我们的经验与60例WM患者，侧重于他们的临床表现，对治疗的反应，并可能识别预后因素。在这些患者中，70%表现为疲劳，22%观察到淋巴结肿大，18%脾肿大，13%肝肿大，6%累及淋巴结部位。17%的患者有出血倾向，17%的患者有感染，12%的患者有高粘滞综合征，25%的患者有心力衰竭。IgM水平中位数为30 g/l，20%的病例伴有低白蛋白血症，27%伴有低丙种球蛋白血症，15%伴有多克隆高丙种球蛋白血症，78%伴有κ轻链限制，54%伴有Bence-Jones蛋白尿。贫血常见（85%），其次是白细胞增多（18%）、淋巴细胞增多（12%）、白细胞减少（10%）和血小板减少（10%）。冷球蛋白血症和自身免疫性溶血性贫血发生率为5%。除两例外，所有病例均累及骨髓。在50名最初接受间歇性口服苯丁酸氮芥治疗的患者中，46名（92%）有反应。中位总生存期为108个月。与不良预后相关的因素为年龄大于或等于65岁（p=0.06）、存在淋巴结病（p=0.06）、骨髓浸润大于或等于50%（p=0.007）、国际预后指数（IPI）大于或等于3（p=0.0001）和Morel评分系统（p=0.04）。总之，我们发现在这一系列WM患者中，苯丁酸氮芥是一种有效的治疗方法，并且年龄、淋巴结病、骨髓浸润百分比、IPI和Morel评分系统等参数具有预后意义。
Waldenstrom's macroglobulinemia (WM) is a lymphoplasmacytic lymphoma characterized by the presence in patients' serum of an I-M monoclonal component. We report on our experience with 60 WM patients, focusing on their clinical findings, response to treatment, and the possible identification of prognostic factors. Of these patients, 70% presented with fatigue, and lymphadenopathy was observed in 22%, splenomegaly in 18%, hepatomegaly in 13%, and extranodal site of involvement in 6%. Bleeding tendency was seen in 17%, infections in 17%, hyperviscosity syndrome in 12%, and cardiac failure in 25% of the patients. The median of IgM levels was 30 g/l with hypoalbuminemia in 20% of cases, hypogammaglobulinemia in 27%, polyclonal hypergammaglobulinemia in 15%, kappa light-chain restriction in 78%, and Bence-Jones proteinuria in 54%. Anemia was frequent (85%), followed by leukocytosis (18%), lymphocytosis (12%), leukopenia (10%), and thrombocytopenia (10%). Cryoglobulinemia and autoimmune hemolytic anemia were encountered in 5%. In all cases but two, bone marrow was involved. Of 50 patients initially treated with intermittent oral chlorambucil, 46 (92%) responded. Median overall survival was 108 months. Factors associated with adverse prognosis were age greater than or equal to65 years (p=0.06), presence of lymphadenopathy (p=0.06), bone marrow infiltration greater than or equal to50% (p=0.007), international prognostic index (IPI) greater than or equal to3 (p=0.0001), and Morel's scoring system (p=0.04). Concluding, we found in this series of WM patients that chlorambucil is an effective treatment and that the parameters of age, lymphadenopathy, percentage of bone marrow infiltration, IPI, and Morel's scoring system carry prognostic significance.