Paraproteinemia

Paraproteinemia is a condition in which there are substances called paraproteins in the blood. In particular, a special monoclonal immunoglobulin and corresponding immunoglobulin light chains are increasingly present in the blood.

Paraproteinemia

What is paraproteinemia?

Paraproteinemias are also known as monoclonal gammopathies. They describe the occurrence of a homogeneous immunoglobulin in human blood. In the past, paraproteinemia that was not associated with multiple myeloma or other clonal lymphoproliferative diseases was referred to as ‘benign gammopathy’ or ‘benign paraproteinemia’. See ezhoushan for What does CDA Stand for.

However, in recent years, the term ‘monoclonal gammopathy of undetermined significance’ has become established among medical professionals for these diseases. The abbreviation for this designation is MGUS and is used in many cases. MGUS is defined as a disease in which people have a prolonged presence of monoclonal immunoglobulin in their urine or serum.

This immunoglobulin must be present in a constant concentration and the disease must be asymptomatic. The monoclonal immunoglobulin is formed in the bone marrow. Certain plasma cells are responsible for the production of the substance, which proliferate slowly and do not exhibit any malignant behavior. Very often, the disease can only be diagnosed after a long follow-up.

Causes

Paraproteinemias are usually associated with various other diseases and symptoms. Depending on the disease in question, the causes for the occurrence of paraproteinemia also differ. Subgroups were formed to get a better overview of common clinical pictures and their causes.

These groups each refer to a protein whose concentration exceeds the normal value for various reasons. The subgroups usually refer to multiple myeloma, osteoclastic multiple myeloma, IgG deposition disease and Waldenstrom’s disease. Other subgroups include monoclonal gammopathy of unknown significance and heavy chain disease. It should be noted that some of these diseases belong to the so-called non-Hodgkin lymphomas.

Symptoms, Ailments & Signs

The symptoms and signs of paraproteinemia manifest themselves in many ways and vary from patient to patient and depending on the underlying disease. A typical feature of paraproteinemia, for example, is the hyperviscosity of the blood.

The viscosity is more pronounced than usual due to the increased number of proteins in the blood. As a result of such hyperviscosity, other ailments are possible. These include, for example, neuropathies, amyloidosis and blood clotting disorders. In addition, so-called cold agglutinins are sometimes detected at the same time.

At the same time, in the context of paraproteinemia, there is in many cases a lack of normal immunoglobulins. This can cause an increased susceptibility to infection in the affected persons. In principle, it is a paraproteinemia when the plasma cell content in the bone marrow cells is higher than 30 percent.

The term paraproteinemia is also used when a tumor composed of plasma cells is detected in a tissue biopsy.

Diagnosis & course of disease

With regard to the diagnosis of paraproteinemia, there are various options to choose from. In principle, every detected paraproteinemia should initially be regarded as multiple myeloma or plasmacytoma until this suspicion is refuted. It is therefore necessary to quantitatively determine the immunoglobulins both in the urine and in the serum.

In addition, the immune fixation must be analyzed. In addition, the blood count and in particular the substances calcium and creatinine in the serum must be examined. X-rays include images of the spine, skull and pelvis. In certain cases, an MRI scan of the spine is indicated.

Bone marrow may require a biopsy. Patients must undergo regular check-ups to continuously monitor existing paraproteins and other parameters. Bone marrow findings are often checked annually, especially in younger patients.

All pain that occurs in the musculoskeletal system must be examined using imaging methods. Urine samples are particularly suitable for diagnosing paraproteinemia. This is because it is easy to determine if the protein content is too high. Paraproteinemia can be assumed as soon as the concentration of certain proteins in the urine rises to more than one gram per 24 hours.

Complications

Because of paraproteinemia, those affected may suffer from various ailments. As a rule, however, there are disturbances in blood clotting, so that the blood is very viscous. Furthermore, the patient’s immune system is also significantly weakened, making them more susceptible to various infections and diseases. The patient’s lymph nodes are often swollen with paraproteinemia and those affected feel ill and exhausted.

In the worst case, it can also lead to a tumor. If this remains undetected and not treated, it often leads to the death of the person concerned. Paraproteinemia can be treated with chemotherapy or other medications. However, there are various side effects that can have a very negative impact on the life of those affected.

Radiation or stem cell transplantation can also relieve paraproteinemia. However, the tumor must be surgically removed. This may also reduce the life expectancy of the person affected. Furthermore, many patients are dependent on psychological treatment if they develop depression or other psychological problems.

Treatment & Therapy

In the case of paraproteinemia, the focus is basically on the therapy of the respective symptoms. The treating specialist decides on the therapeutic measures depending on the individual case. The aim is to eliminate the cause of the pathological increase in protein. Various treatment options are available for this purpose.

These include, for example, chemotherapy, bisphosphonate treatment or immunomodulation. Radiation therapy, stem cell transplantation and surgical interventions are also possible. The protein-producing tumor is surgically removed.

The prognosis of the disease depends on various criteria. If, for example, the amount of paraprotein increases continuously, this worsens the prognosis. In rare cases, affected patients develop Waldenstrom’s disease, malignant non-Hodgkin’s lymphoma or amyloidosis.

Outlook & Forecast

The prognosis for paraproteinemia is highly dependent on the underlying disease that caused it. Most patients are diagnosed with chronic diseases that ultimately lead to changes in blood clotting. Since the underlying diseases often cannot be cured, the future prospects for the development of paraproteinemia are unfavorable. Rather, those affected need lifelong drug treatment in order not to trigger acute health conditions.

In addition, complications can be expected. Patients with paraproteinemia often have an increased susceptibility to infections. The organism is weakened overall and can no longer react adequately to pathogens as usual. With a very unfavorable development, the patient develops a tumor. As a result, there is a potential threat to the probable life expectancy and the ability to cope with everyday life is severely restricted. Cancer therapy is necessary to alleviate the existing symptoms. Despite all efforts, the tumor disease ends in premature death in most patients.

When making a prognosis, it must also be taken into account that the emotional stress caused by the general state of health can lead to subsequent psychological disorders. These also lead to a difficult further development overall and can be very tedious. In most cases, this also leads to a further deterioration in physical health.

Prevention

Concrete measures to prevent paraproteinemia are not known according to the current state of medical research. For this reason, a suitable specialist should be consulted at the slightest symptoms or signs of the disease. Because the earlier the diagnosis is made, the more favorable the prognosis for paraproteinemia can be.

Aftercare

In the case of paraproteinemia, the aftercare measures are very limited in most cases. Ideally, the person affected should consult a doctor at an early stage so that other complications and symptoms do not arise for the person concerned. The sooner a doctor is consulted, the better the further course of the disease.

Most patients rely on removal of the tumor. Bed rest should be observed after surgery. Efforts or stressful physical activities should be avoided in order not to unnecessarily burden the body. Regular checks and examinations should also be carried out by a doctor in order to quickly identify and remove other tumors.

It is not uncommon for the psychological support of one’s own family to be very important, whereby loving and intensive conversations in particular can have a positive effect on the further course of the disease. The further course of paraproteinemia depends heavily on the time of diagnosis, so that a general prediction is usually not possible. This disease may also limit the life expectancy of those affected.

You can do that yourself

This diagnosis requires a thorough examination of the patient to determine what is causing the paraproteinemia. This underlying condition may be a tumor and needs to be treated accordingly. The check-ups that are due as the disease progresses should also be observed and any additional physical complaints should be taken seriously.

Patients suffering from paraproteinemia usually feel tired and exhausted. There is also an increased susceptibility to infection. In order to minimize these symptoms, an appropriate lifestyle is helpful. This lifestyle is made up of various components. On the one hand, there is the diet, which should consist of fresh ingredients and be rich in vitamins and fiber. Any existing excess weight should be gently reduced. In order to be able to flush toxins out of the body, it is also advisable to drink a lot. Still mineral water, teas and thin juice spritzers have proven their worth here.

According to the latest research, a healthy diet also has a positive influence on any existing depression. Since paraproteinemia is a very distressing diagnosis, additional concomitant therapeutic therapy may be indicated. A paraproteinemia patient also benefits from a regular sleeping and waking cycle. He should avoid alcohol and nicotine and instead exercise regularly. Long walks in the forest or bike rides in the fresh air have proved their worth here.