Waldenström macroglobulinemia manifests mainly with fatigue secondary to chronic anemia, bleeding through the nose and gums, and enlargement of the liver and spleen.
Waldenström macroglobulinemia is a cancer of the cells of the blood system. It’s characterised by the proliferation of B lymphocytes (cells which are a part of white blood cells and are liable for antibodies) with excessive production of immunoglobulin or monoclonal M antibody (derived from a single cell type) and infiltration of the bone marrow.
It’s a chronic and rare disorder. Its incidence is estimated to be 3 patients per million people per yr.
Its clinical manifestations are varied, starting from nonspecific signs to those related to changes within the blood as a consequence of exaggerated antibody production. Fatigue, related to chronic anemia, is probably the most common symptom.
Bone marrow, blood cells, and causes
Bone marrow is situated contained in the bones and is the place where blood cells (red blood cells, white blood cells, and platelets) are produced.
B lymphocytes are a part of the white blood cells and are liable for producing antibodies. Antibodies are proteins liable for directly attacking foreign agents that invade the body and signal an alarm.
Within the case of Waldenström macroglobulinemia, there’s an exaggerated proliferation of B lymphocytes (plasma cells) that produce monoclonal antibodies of sophistication M.
Thus far, the particular reason behind Waldenström macroglobulinemia isn’t yet known. It’s considered a sporadic disease.
It’s related to familial predisposition in as much as 20% of cases (related to first-degree relatives) and with specific chromosomal deletions (present in cases where there’s no familial predisposition).
Symptoms of Waldenström macroglobulinemia
Waldenström macroglobulinemia is a rare chronic disorder. Its incidence is low, as mentioned above, but is higher in white people.
It mostly develops between the sixth and seventh decade of life. The mean age of diagnosis is 65 years and it predominates in males over females.
The estimated survival of Waldenstrom macroglobulinemia is just 5 to six years.
The symptoms are varied, nonetheless, probably the most common are the next:
- Chronic anemia
- Nasal and gum bleeding
- Presence of lymphadenopathy
So far as non-specific signs are concerned, they vary from fever without focus, weight reduction greater than 10%, and profuse night sweats, in addition to general symptoms (malaise, fatigue, anorexia).
As a result of the exaggerated production of immunoglobulin M within the bone marrow, the blood becomes denser, so the flow is slower and there’s a greater probability of obstruction. That is often called hyperviscosity syndrome.
As well as, this results in enlargement of the lymph nodes (lymphadenopathy), liver (hepatomegaly) and spleen (splenomegaly). There’s also 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 macroglobulinemia, hyperviscosity syndrome creates symptoms equivalent to the next:
- Nasal bleeding
- Vision disturbances
- Raynaud’s disease
- Renal insufficiency
- Retinal hemorrhage
Diagnosis of Waldenström macroglobulinemia
The diagnosis of Waldenström macroglobulinemia is made by the detection of M antibodies in blood with a concentration greater than 3 grams per liter. As well as, plasma cell proliferation should be demonstrated on bone marrow biopsy.
A complementary immunofixation test is performed to confirm that the M antibody is derived from a single cell type (monoclonal).
Hemoglobin levels of 10 grams per deciliter could also be present initially, representing mild anemia. This chronic form is well tolerated by patients.
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Treatment will rely upon symptoms and evolution
Waldenström macroglobulinemia tends to progress very slowly, over a few years, before requiring treatment. That’s why in some patients (in the event that they don’t have any symptoms or significant changes) no treatment is beneficial.
When treatment is resorted to, monoclonal antibodies or alkylating agents are used alone or in association with chemotherapy. The latter is beneficial when there are various monoclonal antibody-producing cells within the bone marrow or there are various antibodies within the blood.
In cases where the hyperviscosity syndrome of Waldenström macroglobulinemia is severe, plasmapheresis could also be used. On this procedure, blood is extracted, processed in a machine that removes the abnormal proteins after which reintroduced into the body. In this manner, the antibodies are removed and the viscosity is reduced.
Although there’s little experience, some patients may profit from bone marrow transplantation.
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A pathology that’s difficult to diagnose
If Waldenström macroglobulinemia is suspected, a specialist physician (hematologist) must be consulted for diagnosis. The initial manifestations are very unspecific, so it’s common for several kinds of cancer to be taken into consideration before the definitive verdict.
Although the estimated survival after diagnosis seems short, when there aren’t any symptoms the evolution takes several years longer.
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