Defined as a disease of the proliferating blood in the bone marrow, Waldenström disease is characterized by the presence of white blood cells of a particular type, B lymphocytes which transform into plasma cells and synthesize a monoclonal antibody in large quantities.

Waldenström’s disease is linked to the proliferation of lymphoid cells (B lymphocytes) in the bone marrow, and more rarely in the ganglia and spleen. At the same time, these abnormal lymphocytes turn into mast cells which produce an antibody called monoclonal immunoglobulin M (IgM). Produced in sometimes very large quantities, this IgM circulates in the blood.

It is therefore a cancerous type disease of the bone marrow, that is to say the tissue contained in the bones where all the blood cells are produced. Its evolution is slow over several years and sometimes does not give any symptoms, especially at the beginning. 

A rare disease

It is a rather rare disease (180 to 300 cases in France), which preferably affects men. The diagnosis is most often made around the age of 63. Its cause is unknown. There are, however, family forms, especially in siblings.

Often, Waldenström’s disease does not initially present symptoms (asymptomatic) and progresses very gradually.

When symptoms do appear, it is most often:

• progressive fatigue (asthenia);
• the appearance of lymph nodes in various areas of the body in the neck (cervical), armpits (axillary), groin area (inguinal);
• the presence of a large spleen (splenomegaly) on physical examination;
• small hemorrhages in the skin or mucous membranes;
• pallor of the skin and mucous membranes associated with anemia;
• shortness of breath related to anemia;
• hyperviscosity of the blood (thick blood) which causes headache, dizziness and ringing in the ears (tinnitus);
• tingling in the feet and hands;
• a decrease in sensitivity of the skin, in particular at the extremities;
• muscle cramps and pain.

Then, when the disease progresses, all these symptoms can worsen in a context of deterioration of the general condition (AEG) with weight loss, severe asthenia, anorexia, hemorrhagic phenomena in various places of the body.

Several laboratory tests are prescribed to find and confirm a diagnosis of Waldenström’s disease:

Blood Formula Count (BFS) 

This is a routine test and may show anemia, red blood cells clumped together in coils, a low number of platelets (thrombocytopenia), and a slight increase in some white blood cells, lymphocytes.

A sedimentation rate (VS) 

As well as a very high C reactive protein (CRP) both indicating inflammation of the body.

Electrophoresis of proteins

Allowing to search for immunoglobulins circulating in the blood and for which a peak of immunoglobulins is found (Kappa immunoglobulin).

And myelogram 

It will be prescribed if this peak is confirmed. This test involves taking a small part of the bone marrow to look for a marrow invaded by lymphoid cells (lymphocytes and plasma cells). It is this last examination which will confirm the diagnosis of Waldenström’s disease.

Other biological abnormalities are often found such as proteins in the urine (proteinuria) or disorders of coagulation factors.

Once the diagnosis is confirmed, treatment will involve specific chemotherapy with a combination of anticancer molecules. Cortisone can also be prescribed to reduce the overall inflammatory syndrome and reduce bleeding phenomena.

Filtration of the plasma (liquid in which the figured elements of blood, red blood cells, white blood cells, platelets…) bathe to remove excess immunoglobulins (plasmapheresis).

Finally, the spleen, which contains a lot of red blood cells, can be removed (splenectomy) and radiotherapy sessions can be performed if necessary.