Waldenström macroglobulinemia with extramedullary involvement at initial diagnosis portends a poorer prognosis

Journal of Hematology & Oncology

Ken H. Young⁵  L. Jeffrey Medeiros³  Zijun Y. Xu-Monette³  Roberto N. Miranda³  Donna Weber¹  C. Cameron Yin³  Muzaffar Qazilbash²  Shaoying Li³  Jatin Shan¹  Sheeba K. Thomas¹  Meifeng Tu³  Sanam Loghavi³  Xiaoxiao Wang³  Robert Z. Orlowski¹  Qing Ye³  Xin Cao⁴ 

[1] Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX, USA;Department of Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, TX, USA;Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston 77030, TX, USA;Department of Hematology, the Affiliated Hospital of Nantong University, Nantong, China;The University of Texas School of Medicine, Graduate School of Biomedical Sciences, Houston, TX, USA

关键词: Treatment;    Prognosis;    Extramedullary;    Lymphoplasmacytic lymphoma;    Waldenström macroglobulinemia;   

Others  :  1219924
DOI  :  10.1186/s13045-015-0172-y

 received in 2015-04-21, accepted in 2015-06-11,  发布年份 2015

【 摘 要 】

Background

The prognostic importance of extramedullary involvement in patients with Waldenström macroglobulinemia (WM) at diagnosis and treatment options for these patients has not been well evaluated. In this study, we investigated the clinical manifestations, biological features, and effect of first-line therapy on the outcome of WM patients diagnosed with extramedullary involvement (EMWM) vs those with only bone marrow involvement (BMWM).

Methods

We analyzed the clinical data of 312 WM patients diagnosed with EMWM (n = 106) and BMWM (n = 206) at The University of Texas MD Anderson Cancer Center from 1994 to 2014. EMWM was confirmed by biopsy, positron emission tomography–computed tomography, or magnetic resonance imaging, and clinical laboratory analyses.

Results

Characteristics associated with EMWM were male sex (P = 0.027), age younger than 65 years (P = 0.048), presence of B symptoms (P < 0.001), high serum beta-2 macroglobulin (P < 0.001) level, low serum albumin level (P = 0.036), and cytogenetic abnormalities (P = 0.010). Kaplan-Meier survival analysis results showed that EMWM patients had a significantly shorter median overall survival (P < 0.001) and progression-free survival (PFS) (P < 0.001) than did BMWM patients. Chemotherapy combined with targeted therapy improved PFS for BMWM patients (P = 0.004) but not for EMWM patients. Additionally, initial treatment with rituximab significantly improved the PFS of BMWM patients (P = 0.012) but had no effect on EMWM patients. However, EMWM patients treated with nucleoside analogs attained a better PFS than those who did not (P = 0.021).

Conclusions

We show that extramedullary involvement at diagnosis is an adverse prognostic factor in WM patients and that first-line therapy with nucleoside analogs improved PFS for patients with EMWM. The study provides unique clinical and treatment observations in subtypes of WM patients.

【 授权许可】

   
2015 Cao et al.

【 图 表 】

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