Fast Facts for Patients: Waldenström Macroglobulinemia

S. D'Sa

Description

Waldenström macroglobulinemia (shortened to WM) is a rare blood cancer. WM usually progresses slowly; some people do not show symptoms for several years after diagnosis. A person who does not have symptoms usually does not need treatment, but active monitoring is essential so that treatment can be started as soon as it is needed. Although there is no cure for WM, different treatment options can keep the disease under control for many years in a lot of people.  Eventually, the treatments tend to lose their effect. New therapies are being tested in clinical trials across the world, with promising results.

Table of Contents:

• What is Waldenström macroglobulinemia? How will WM affect me? Who is in my care team? What tests will I need to have? Common feelings when diagnosed

• Helping yourself

• Active monitoring

• Starting treatment

• Types of treatment

• What are supportive treatments? How do I know if treatment has worked? Follow-up after treatment

• When WM comes back

• Research and new treatments

• Understanding WM 

Sujets

• Medical
• Médecine
• Oncology

Informations

• Publié par: S. Karger AG
• Date de parution: 17 juin 2022
• Nombre de lectures: 0
• EAN13: 9783318071443
• Langue: English
• Poids de l'ouvrage: 3 Mo

Extrait

Note: Abbreviations and medical terms are explained in the Glossary on page 45 .
First, the facts

Waldenstr m macroglobulinemia (shortened to WM) is a rare blood cancer.
WM usually progresses slowly; some people do not show symptoms for several years after diagnosis.
A person who does not have symptoms usually does not need treatment, but active monitoring is essential so that treatment can be started as soon as it is needed.
Although there is no cure for WM, different treatment options can keep the disease under control for many years in a lot of people.
Eventually, the treatments tend to lose their effect. New therapies are being tested in clinical trials across the world, with promising results.
This book provides the information you need to have an informed discussion with your healthcare professional and to help you choose how you want to manage your condition. Spaces have been provided to help you keep notes on your symptoms and concerns and record any questions you may have.

My main concerns
Make a note of anything you want to discuss with your doctor here
What is Waldenstr m macroglobulinemia?
How will WM affect me?
Who is in my care team?
What tests will I need to have?
Common feelings when diagnosed
Helping yourself
Active monitoring
Types of treatment
What are supportive treatments?
Follow-up after treatment
When WM comes back
Research and new treatments
Understanding WM
Glossary and abbreviations
What is Waldenstr m macroglobulinemia?
WM is a type of non-Hodgkin lymphoma known as lymphoplasmacytic lymphoma (LPL). It is a rare blood cancer in which abnormal cells build up in the bone marrow and other places.
WM most commonly affects people over 60 and slightly more men than women.
To understand WM it helps to get to know some of the terms that your doctor may use and to learn a bit about the biology of the disease. Understanding what causes WM and how the disease develops will help you to ask the right questions and make good treatment choices.
Turn to page 39 to find out more about:
normal blood cell production and what goes wrong in WM
a paraprotein called IgM
a condition called MGUS that can develop into WM
the gene mutations that produce abnormal proteins in your body.
The abbreviations and medical terms used throughout this book are also explained in the Glossary and abbreviations on page 45 .
How will WM affect me?
Symptoms vary from person to person depending on how the disease develops. Not everyone gets all of the symptoms. Even after diagnosis, you may not get any symptoms for many years.
Symptoms may develop because of:
disrupted production of normal blood cells
thickening of the blood because of high levels of IgM paraprotein, an abnormal immunoglobulin (antibody); see pages 40 and 42 .
IgM paraproteins mistakenly targeting tissues and organs
the IgM becoming sticky or fragmented
the IgM coating nerve cells and causing damage (peripheral neuropathy).
Over time, LPL cells fill up the bone marrow or collect in the lymph nodes or the spleen (and, rarely, in other places in the body).
Effects of having fewer healthy blood cells
Having fewer red blood cells leads to anemia, which can cause tiredness, weakness and breathlessness. This is linked with fatigue, an extreme form of tiredness that is the most common symptom of WM. A lack of white blood cells weakens the immune system, so you may tend to develop infections. A lack of platelets leads to a tendency to bruise or bleed easily, as platelets are important in blood clotting.
B symptoms
Fevers, night sweats and weight loss may be a feature of later-stage WM. You might hear them referred to as B symptoms. They happen when the LPL cells build up to the extent that their metabolic activity becomes physically noticeable through these symptoms. Tell your doctor if you start to notice these symptoms - for example, if you need to change nightclothes regularly or you have lost a significant amount of weight. You may need to start treatment.
Swellings and lumps
In about a quarter of patients with WM, lymph nodes and/or the spleen may enlarge; you might notice swollen glands. Rarely, a swollen spleen can be uncomfortable or painful.
LPL cells may collect in body cavities, causing soft tissue masses (lumps) or a build up of fluid in the chest (pleural effusion). Rarely, the cells may build-up in the skeleton, causing bone pain. If these symptoms appear, the diagnosis is confirmed by taking scans and analyzing samples of tissue from a biopsy.
Some of the possible effects of WM
The effects of WM vary from person to person. You may experience some or none of these effects.

Anti-MAG neuropathy
IgM paraproteins may mistakenly target tissues and organs in the body. In anti-MAG (myelin-associated glycoprotein) neuropathy, the IgM paraprotein damages the axons of nerves (neurons) or the myelin that insulates them. This can lead to numbness or tingling in the hands and feet or problems with balance if the nerve damage is in the limbs (peripheral neuropathy).
It is important to mention any of these symptoms to your doctor, especially if they are getting worse over time.
You may need tests to examine your nervous system in more detail: a scan of your brain or spinal cord, a lumbar puncture to look for signs of inflammation, nerve conduction studies to see how well your nerves are conducting electrical impulses or perhaps a nerve biopsy (under a local anesthetic).

Hyperviscosity syndrome
As the graph shows, once blood IgM paraprotein levels increase past a certain point, the blood becomes much thicker or more viscous. This is called hyperviscosity syndrome.
Symptoms include bleeding from the nose and mouth, headaches, blurred or loss of vision and dizziness. These symptoms are more likely to occur if your IgM level is over 40 g/L. Your doctor will recommend treatment if you have symptoms.
A test for blood thickness (serum or plasma viscosity) can be done in specialist laboratories.

Bing-Neel syndrome
Very occasionally, LPL cells build up in the central nervous system (CNS), resulting in Bing-Neel syndrome. The symptoms are varied, but may include headaches, seizures, weakness of the facial or limb muscles, double vision, personality change and memory loss. Special tests such as brain scans and sampling of the cerebrospinal fluid by a lumbar puncture are needed to identify the cells. This rare complication can be treated if recognized promptly but requires special treatments.
Cold agglutinin disease
Cold temperatures may trigger IgM to act as a bridge between red blood cells, causing them to stick together in the cooler parts of the body, such as the hands and feet, the tip of the nose and the ear lobes. This is called agglutination and the condition is called cold agglutinin disease (CAD).
The affected areas have poor blood circulation, especially when it is cold: color changes and ulcers may develop as the skin breaks down. CAD also results in the breakdown of red blood cells (hemolytic anemia) due to activation of a part of the immune system called complement.