Familial Disease Predisposition Impacts Treatment Outcome in Patients With Waldenstr?m Macroglobulinemia

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• 作者：Steven P. Treon¹ ; ^{steven_treon@dfci.harvard.edu} ; Christina Tripsas¹ ; Christina Hanzis¹ ; Leukothea Ioakimidis¹ ; Christopher J. Patterson¹ ; Robert J. Manning¹ ; Patricia Sheehy¹ ; Barry Turnbull² ; Zachary R. Hunter¹
• 关键词：Bortezomib ; Familial predisposition ; Rituximab ; Waldenströ ; m macroglobulinemia
• 刊名：Clinical Lymphoma Myeloma and Leukemia
• 出版年：2012
• 出版时间：December, 2012
• 年：2012
• 卷：12
• 期：6
• 页码：433-437
• 全文大小：531 K

文摘

Background

We examined the impact of familial predisposition on treatment outcome in 135 patients with Waldenstr?m macroglobulinemia (WM), 26.7 % of whom had first- or second-degree relatives with a B-cell lymphoproliferative disorder.

Patients and Methods

All patients were rituximab naive and received a rituximab-containing regimen. There were no significant differences in baseline characteristics between cohorts.

Results

Overall response (93.9 % vs. 75.0 % ; P = .029) and complete response/very good partial response (CR/VGPR) (23.2 % vs. 16.7 % ; P < .0001), time to progression (TTP) (45.5 vs. 21 months; P = .015) and time to next therapy (TTNT) (50.0 vs. 33.0 months; P = .024) favored patients with sporadic WM. By multivariate analysis, familial predisposition was an independent marker for disease progression (hazard ratio, 0.554). Patients with familial but not sporadic disease exhibited better responses, including CR/VGPR attainment (P = .0006) and a trend for longer progression-free survival (> 33 vs. 20.6 months; P = .08), with bortezomib-containing therapy.

Conclusion

The findings convey that familial predisposition is an important determinant of treatment outcome in WM. Prospective studies to confirm these observations are needed.