How is bulky symptomatic Waldenstrom macroglobulinemia (WM) treated?

Updated: Aug 06, 2020
  • Author: Joseph M Tuscano, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Answer

Bulky symptomatic disease is characterized by one or more of the following:

  • Bulky adenopathy
  • Symptomatic hepatosplenomegaly
  • Cytopenias
  • Hyperviscosity
  • Constitutional symptoms

See Table 1, below, for common regimens that have been used and outcome data. Bendamustine/rituximab is the preferred initial therapy for most patients, while ibrutinib/rituximab is an acceptable alternative for elderly patients who cannot tolerate chemotherapy. [4] Bortezomib should be avoided in patients with baseline neuropathy. Nucleoside analogs such as fludarabine or cladribine pose a risk of stem cell damage and leukemogenesis and should not be used as initial therapy in patients who are candidates for stem cell transplantation (SCT). [5]

 

Table 1. Common regimens used to treat bulky symptomatic Waldenstrom macroglobulinemia (Open Table in a new window)

Regimen

Dose/Schedule

Study Results

BR

Bendamustine 90 mg/m2 IV days 1-2

Rituximab 375 mg/m2 IV day 1

Repeat 28-day cycle for 4-6 cycles

Rummel et al [6] : 41 treatment-naive patients; ORR 95%

DRC

Dexamethasone 20 mg IV day 1

Rituximab 375 mg/m2 IV day 1

Cyclophosphamide 100 mg/m2 PO BID days 1-5

Repeat 21-day cycle for 6 cycles

Kastritis et al [7] : 72 treatment-naive patients;

ORR 83%, CRR 7%

BDR

Bortezomib 1.3 mg/mIV days 1, 4, 8, 11 during cycle 1 followed by 1.6 mg/mon days 1, 7, 15, and 22 for cycles 2-5

Dexamethasone 40 mg IV weekly starting day 1 of cycle 2

Rituximab 375 mg/m2 IV weekly starting day 1 of cycle 2

5 cycles total; 21 days for cycle 1; 35 days for cycles 2-5

Gavriatopoulou et al [8] : 59 treatment-naive patients;

ORR 85%, CRR 3%

Rituximab

Rituximab 375 mg/m2 IV once weekly x 4 weeks

Gertz et al [9] : 69 treatment-naive and previously treated patients; ORR 27.5%, CRR 0%

Ibrutinib

Ibrutinib 420 mg PO once daily until disease progression

Treon et al [10] : 30 treatment-naive patients; ORR 100%, CRR 0%

Treon et al [11] : 63 previously treated patients; ORR 90.5%

Ibrutinib/rituximab

Ibrutinib 420 mg PO once daily until disease progression

Rituximab 375 mg/m2 IV once weekly during weeks 1-4 and weeks 17-20

Dimopoulos et al [12] : 150 treatment-naive and previously treated patients; ORR 92%, CRR 3%

Acalabrutinib

Acalabrutinib 100 mg PO BID until disease progression

Owen et al [13] : 106 treatment-naive and previously treated patients; ORR 93%, CRR 0%

Cladribine/rituximab

Cladribine 0.1 mg/kg SC days 1-5

Rituximab 375 mg/m2 IV day 1

Repeat 28-day cycle for 4 cycles

Laszlo et al [14] : 29 treatment-naive and previously treated patients; ORR 90%, CRR 24%

Fludarabine/rituximab

Fludarabine 25 mg/m2 IV days 1-5

Rituximab 375 mg/m2 IV day 1

Repeat 28-day cycle for 4-6 cycles

Peinert et al [15] : 27 treatment-naive and previously treated patients; ORR 90%, CRR 3%

FCR

Fludarabine 25 mg/m2 IV days 1-3

Cyclophosphamide 250 mg/m2 IV days 1-3

Rituximab 375 mg/m2 IV day 1

Primary: Repeat 28-day cycle for 4-6 cycles

May also be given with mitoxantrone 10 mg/m2 on day 1

Tedeschi et al [16] : 43 treatment-naive and previously treated patients; ORR 79%, CRR 11.6%

ORR = overall response rate; CRR = complete response rate


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