NCCN Guidelines for Patients
Waldenström’s Macroglobulinemia, Version 1.2017
This drug can cause an IgM flare. The level of IgM
goes up due to large amounts being released from
dying cells. This flare will cause hyperviscosity and
the symptoms that come with it. Your doctor may
recommend plasmapheresis before you receive this
drug. This depends on whether your IgM level is very
high or a test for serum viscosity shows your blood is
Rituximab also increases your chances for tumor
lysis syndrome. This syndrome causes problems
in the blood from cells dying and leaving waste.
Other problems include heart issues or a blockage
and holes in your gut. Serious infections, such as
progressive multifocal leukoencephalopathy, are very
If you who can’t take rituximab, your doctor can
offer ofatumumab. This drug also targets the CD20
antigen. It can briefly increase IgM in the blood. Your
doctor will continue to check your IgM level if you
receive targeted therapy.
Bortezomib is another targeted therapy used to
treat WM. It is very active in the treatment of WM.
It works by a number of mechanisms, one of which
stops the proteasome in the cell. The proteasome
is the machinery needed to dispose of unwanted
proteins in the cell. Bortezomib stops the cell division
and causes cell death. It can be given alone, with
rituximab, or with rituximab and dexamethasone.
Bortezomib is a liquid that is slowly injected into a
vein for a certain period of time. It can also be given
as an injection under the skin (subcutaneous). The
doctor will decide on the amount given, how long it is
given, and how often you get this drug.
Common side effects of bortezomib are:
Extreme tiredness (fatigue)
Not wanting to eat
Nausea and vomiting
Low blood cell counts
Nerve damage (neuropathy)
This drug can cause a certain type of neuropathy
called peripheral neuropathy. This type of neuropathy
affects the hands and feet. It can begin with
sensitivity to cold, pain, burning, and numbness. If
someone already has this neuropathy, this drug can
make it worse.
Bortezomib can also reactivate the herpes zoster
virus. This virus can cause painful blisters or a rash
on the skin. Your doctor can give medication to
prevent the virus from reactivating.
Ibrutinib interferes with the BTK (
inase). BTK is activated by mutated MYD88. People
with CXCR4 mutations show lower response rates
and delayed responses to ibrutinib. This molecule of
the B-cell helps the cell survive by sending signals.
This drug stops the signals so the cell can’t grow or
Ibrutinib is given alone. It comes in pill form and is
taken by mouth. It is usually taken once a day. Your
doctor or pharmacist can answer any questions you
have about the dose or time you should take this