Blood Res.  2018 Jun;53(2):117-122. 10.5045/br.2018.53.2.117.

Impact of rituximab and half-dose CHOP as primary therapy for untreated symptomatic Waldenström Macroglobulinemia: review of a combined regimen of rituximab with an alkylating agent

• Sekiguchi
• Hamano A
• Kitagawa T
• Kurihara Y
• Ito K
• Kurimoto M
• Watanabe K
• Hirano K
• Noto S
• Yamada K
• Takezako N

• KMID: 2451029
• DOI: http://doi.org/10.5045/br.2018.53.2.117

Abstract

BACKGROUND
Waldenström Macroglobulinemia (WM) is a rare subtype of indolent B-cell lymphoma, and prospective randomized studies on WM are scarce. The R-CHOP therapy [rituximab (R), cyclophosphamide, hydroxy-doxorubicin, vincristine, and prednisone] is a popular and recommended regimen for primary therapy, prescribed by several treatment guidelines for WM. However, treatment with R-CHOP is accompanied by severe myelosuppression and high rates of peripheral neuropathy. Therefore, we retrospectively evaluated the efficacy and toxicity of half-dose CHOP combined with R as a primary therapy for WM.
METHODS
Patients with untreated symptomatic WM, treated at the Disaster Medical Center between April 2011 and September 2016, were retrospectively analyzed after administration of 6 cycles of half-dose R-CHOP for every 3 weeks. The response, median time to response, best response, progression-free survival, overall survival, and toxicities were evaluated.
RESULTS
Of the 20 WM patients analyzed, 16 (80%) received half-dose R-CHOP without vincristine, and 13 (65%) responded to the treatment. With a median follow-up duration of 26.3 months, the 2-year progression-free survival and 2-year overall survival rates were 70 and 93.3%, respectively. The median time to response and best response were 6 and 9.9 weeks, respectively. Grade 3/4 leukocytopenia, neutropenia, febrile neutropenia, and Grade 1 peripheral neuropathy developed in 32, 37, 0, and 21% of patients, respectively.
CONCLUSION
The half-dose R-CHOP is an effective and well-tolerated primary therapy for WM. To the best of our knowledge, this is the first study reporting the use of a reduced-dose R-CHOP regimen for the primary treatment of WM.

Keyword

Waldenström Macroglobulinemia; R-CHOP; Reduced-dose; Primary therapy

MeSH Terms

Cyclophosphamide
Disasters
Disease-Free Survival
Febrile Neutropenia
Follow-Up Studies
Humans
Leukopenia
Lymphoma, B-Cell
Neutropenia
Peripheral Nervous System Diseases
Prospective Studies
Retrospective Studies
Rituximab*
Survival Rate
Vincristine
Waldenstrom Macroglobulinemia*
Cyclophosphamide
Rituximab
Vincristine

Figure

• 

  Fig. 1 Survival curves showing (A) progression-free survival (PFS). Median PFS was not reached, and the estimated 2-year PFS and 3-year PFS rates were 70% and 60%, respectively; (B) overall survival. The estimated 2-year OS rate was 93.3%; (C) PFS between the high-risk and low/intermediate-risk groups of IPSSWM (P=0.20).

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