Waldenström’s macroglobulinemia is especially manifested by fatigue secondary to chronic anemia, bleeding from the nose and gums, and hepatosplenomegaly.
Waldenstrom’s macroglobulinemia blood cell cancer. It is characterised by the proliferation of B lymphocytes (cells that make up white blood cells and are liable for the production of antibodies) with excessive production of immunoglobulins or M monoclonal antibodies (derived from a single cell type) and bone marrow infiltration.
It is a chronic and rare disease. The incidence is estimated at 3 patients per million people per yr.
Its clinical manifestations are varied, starting from non-specific symptoms to changes within the blood brought on by excessive production of antibodies. The most typical symptom is fatigue, related to chronic anemia.
Bone marrow, blood cells and causes
The bone marrow is contained in the bones and is where blood cells (red cells, white cells and platelets) are made.
B lymphocytes are a part of the white blood cells and are liable for the production of antibodies. Antibodies are proteins liable for directly attacking foreign agents that attack the body and signal the alarm.
In the case of Waldenström’s macroglobulinemia, there’s exaggeration B lymphocytes (plasma cells) that produce class M monoclonal antibodies.
Meet with someone, the particular explanation for Waldenström’s macroglobulinemia shouldn’t be yet known. It is taken into account a sporadic disease.
Up to twenty% of cases are related to a familial predisposition (related to first-degree relatives) and with specific chromosome deletions (present in cases where there isn’t a familial predisposition).
Symptoms of Waldenström’s macroglobulinemia
Waldenström’s macroglobulinemia is a rare chronic disease. Its prevalence is low, as mentioned above, but is higher in Caucasians.
It most frequently develops between the sixth and seventh a long time of life. The average age of diagnosis is 65 years and predominates in men over women.
The estimated survival time for Waldenstrom’s macroglobulinemia is simply 5 to six years.
Symptoms vary, but probably the most common are:
- Chronic anemia
- Thrombocytopenia
- Bleeding from the nose and gums
- The presence of lymphadenopathy
As for non-specific symptoms, they include non-focus fever, weight reduction greater than 10%, and profuse night sweats, as well general symptoms (malaise, fatigue, anorexia).
Due to the excessive production of immunoglobulin M within the bone marrow, the blood becomes thicker, so the flow is slower and there’s a greater risk of obstruction. This is generally known as over-stick syndrome.
In addition, this results in enlarged lymph nodes (enlarged lymph nodes), liver (hepatomegaly) and spleen (enlarged spleen). There can be a decrease within the production of other white blood cells (leukopenia), red blood cells (anemia) and platelets (thrombocytopenia).
Anemia also occurs because M antibodies attach to red blood cells and cause them to rupture.
In Waldenström’s macroglobulinemia, hyperviscosity syndrome causes symptoms reminiscent of:
- headaches
- Epistaxis
- Blurred vision
- Raynaud’s disease
- Kidney failure
- Retinal hemorrhage
Diagnosis of Waldenström’s macroglobulinemia
The diagnosis of Waldenström’s macroglobulinemia relies on: detection of M antibodies within the blood with a concentration greater than 3 grams per litre. In addition, a bone marrow biopsy must show plasma cell proliferation.
A complementary immunofixation test is performed to confirm that the M antibody is from a single cell type (monoclonal).
A hemoglobin level of 10 grams per deciliter could also be present initially, indicating mild anemia. This chronic form is well tolerated by patients.
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Treatment will rely on symptoms and evolution
Waldenstrom’s macroglobulinemia tends to progress very slowlyfor a few years before requiring treatment. Therefore, in some patients (in the event that they don’t have any symptoms or significant changes), no treatment is really helpful.
When treatment is used, monoclonal antibodies or alkylating agents are used alone or together with chemotherapy. The latter is really helpful when there are various monoclonal antibody-producing cells within the bone marrow or when there are various antibodies within the blood.
In cases where Waldenström’s macroglobulinemia hyperviscosity syndrome is severe, plasmapheresis could also be used. In this procedure, blood is drawn, processed in a machine that removes abnormal proteins, after which reintroduced into the body. This removes the antibodies and reduces the viscosity.
Although experience is restricted, some patients may profit from a bone marrow transplant.
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Difficult to diagnose pathology
If Waldenström’s macroglobulinemia is suspectedyou must seek the advice of a specialist doctor (haematologist) as a way to make a diagnosis. The initial symptoms are very non-specific, so it is not uncommon for several sorts of cancer to be considered before a final verdict.
Although the estimated survival time after diagnosis seems short, evolution takes several years within the absence of symptoms.
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