Thalhammer-Scherrer R, Geissler K, Schwarzinger I, Chott A, Gisslinger H, Knöbl P, Lechner K, Jäger U
Department of Medicine I, University of Vienna, Austria.
Ann Hematol. 2000 Oct;79(10):556-9. doi: 10.1007/s002770000185.
Seven patients with macroglobulinemia (six previously untreated, one with minimal pretreatment) were treated with fludarabine (25 mg/m2/day for 5 days, repeated every 4 weeks). The median age was 58 years. The time from diagnosis to treatment with fludarabine was 4.5 months to 175 months (median 32.6 months). The patients received six (n =5), five (n =1), and three (n = 1) courses of fludarabine. One patient showed only a slight decrease of immunoglobulin (Ig) M (from 5,750 mg/dl to 4,700 mg/dl) and no improvement of anemia. Therefore, treatment was stopped after three cycles. In the other six patients, a marked reduction of IgM levels (from 6,140 mg/dl to 1,220 mg/dl median), a normalization of hemoglobin (from 10.8 g/dl to 12.3 g/dl median), a reduction of lymphocyte count (from 1992/>microl to 652/microl median), and a reduction of beta2 microglobulin (from 2.3mg/l to 1.8 mg/l median) were achieved. A 50% IgM reduction was achieved 5.4 months (median) after the beginning of therapy, and the maximum response was observed 17.3 months (median) after the end of treatment. The responses were sustained without further therapy in six patients for 20.8-55.2 months. In one patient, disease progression was observed 12.5 months after the end of therapy. Fludarabine therapy was well tolerated with few side effects. In three patients, febrile episodes occurred. No opportunistic infections were recorded. We conclude that fludarabine is an effective treatment in previously untreated or in minimally pretreated patients with Waldenström's macroglobulinemia.
7例巨球蛋白血症患者（6例未经治疗，1例接受过极少预处理）接受氟达拉滨治疗（25mg/m²/天，共5天，每4周重复一次）。中位年龄为58岁。从诊断到开始使用氟达拉滨治疗的时间为4.5个月至175个月（中位时间32.6个月）。患者接受了6个疗程（n = 5）、5个疗程（n = 1）和3个疗程（n = 1）的氟达拉滨治疗。1例患者仅免疫球蛋白（Ig）M略有下降（从5750mg/dl降至4700mg/dl），贫血无改善。因此，三个周期后停止治疗。在其他6例患者中，IgM水平显著降低（中位值从6140mg/dl降至1220mg/dl），血红蛋白恢复正常（中位值从10.8g/dl升至12.3g/dl），淋巴细胞计数降低（中位值从1992/μl降至652/μl），β2微球蛋白降低（中位值从2.3mg/l降至1.8mg/l）。治疗开始后5.4个月（中位时间）实现了IgM降低50%，治疗结束后17.3个月（中位时间）观察到最大反应。6例患者在未进一步治疗的情况下反应持续了20.8 - 55.2个月。1例患者在治疗结束后12.5个月出现疾病进展。氟达拉滨治疗耐受性良好，副作用较少。3例患者出现发热发作。未记录到机会性感染。我们得出结论，氟达拉滨对未经治疗或接受过极少预处理的华氏巨球蛋白血症患者是一种有效的治疗方法。
