Paraproteinemia is a condition in which so-called paraproteins are present in the blood. In particular, a specific monoclonal immunoglobulin and corresponding immunoglobulin light chains are increased in the blood.
What is paraproteinemia?
Paraproteinemias are also known as monoclonal gammopathies. They describe the presence of a homogeneous immunoglobulin in human blood. In earlier times, paraproteinemia that was not concomitant with multiple myeloma or other clonal lymphoproliferative diseases was referred to as ‘benign gammopathy’ or ‘benign paraproteinemia’. However, in recent years, the term ‘monoclonal gammopathy of undetermined significance’ has become established among physicians for these disorders. The abbreviation for this term is MGUS and is used in many cases. MGUS is defined as a disease in which individuals have a monoclonal immunoglobulin in their urine or serum for a prolonged period of time. This immunoglobulin must be present at a constant concentration and the disease must be asymptomatic. The monoclonal immunoglobulin is formed in the bone marrow. Responsible for its production are certain plasma cells that proliferate slowly and do not exhibit malignant behavior. Very often, the disease can be diagnosed only after a long follow-up.
Causes
Paraproteinemias are usually associated with various other diseases and symptoms. Depending on the particular disease, the causes for the occurrence of paraproteinemia also differ. In order to obtain a better overview of common clinical pictures and their causes, subgroups have been formed. These groups each refer to a protein whose concentration exceeds the normal value for various reasons. The subgroups generally refer to multiple myeloma, osteoclastic multiple myeloma, IgG deposition disease, and Waldenström’s disease. Other subgroups include Monoclonal Gammopathy of Unknown Significance and Heavy Chain Disease. It should be noted that some of these diseases are included in the so-called non-Hodgkin lymphomas.
Symptoms, complaints, and signs
The symptoms and complaints of paraproteinemia manifest in many ways and vary from patient to patient and depending on the underlying disease. For example, a typical feature of paraproteinemia is 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 symptoms are possible. These include neuropathies, amyloidosis and blood coagulation 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 deficiency of normal immunoglobulins. This can cause an increased susceptibility to infections in the affected individuals. In principle, paraproteinemia is present when the content of plasma cells 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 during a tissue biopsy.
Diagnosis and disease progression
With regard to the diagnosis of paraproteinemia, there are several options to choose from. In principle, any paraproteinemia detected should initially be considered a multiple myeloma or plasmacytoma, respectively, until this suspicion is refuted. Thus, it is necessary to quantify the immunoglobulins in both urine and 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-ray examinations include imaging of the spine, skull and pelvis. In certain cases, an MRI examination of the spine is indicated. In the case of bone marrow, a biopsy may need to be performed. Patients must undergo regular check-ups to continuously monitor paraproteins present as well as other parameters. Especially in younger patients, bone marrow findings are often checked annually. All pain occurring in the musculoskeletal system should be investigated using imaging techniques.Urine samples are particularly suitable for diagnosing paraproteinemias. This is because excessive protein levels can be easily detected here. As soon as the concentration of certain proteins in the urine rises to more than one gram per 24 hours, paraproteinemia can be assumed.
Complications
Because of paraproteinemia, affected individuals may suffer from a variety of medical conditions. However, there are usually disturbances in blood clotting, so that the blood is very viscous. Furthermore, the patient’s immune system is also significantly weakened, making him more susceptible to various infections and diseases. It is not uncommon for the patient’s lymph nodes to become swollen in paraproteinemia and for the affected person to feel ill and fatigued. Furthermore, in the worst case, a tumor may develop. If this remains undetected and is not treated, in many cases the affected person will die. Paraproteinemia can be treated with chemotherapy or other drugs. However, there are various side effects that can have a very negative impact on the life of the affected person. Radiation or stem cell transplantation can also alleviate paraproteinemia. However, the tumor must be removed by surgery. This may also reduce the life expectancy of the affected person. Furthermore, many patients rely on psychological treatment if depression or other psychological problems develop.
Treatment and therapy
Basically, the therapy of the respective symptoms is in the foreground in paraproteinemias. The attending specialist decides on the therapeutic measures depending on the individual case. The goal is to eliminate the cause of the pathological increase in protein. For this purpose, various treatment options are available. These include, for example, chemotherapy, bisphosphonate treatment or immunomodulation. Radiation therapy, stem cell transplantation and surgical procedures are also possible. This involves surgical removal of the protein-producing tumor. The prognosis of the disease depends on various criteria. For example, if the amount of paraprotein increases continuously, this worsens the prognosis. In rare cases, affected patients develop Waldenström’s disease, malignant non-Hodgkin’s lymphoma, or amyloidosis.
Outlook and prognosis
The prognosis in paraproteinemia is highly dependent on the precipitating underlying disease. Most patients are diagnosed with chronic diseases that ultimately lead to the change in blood clotting. Since the underlying diseases often cannot be cured, the further outlook for the development of paraproteinemia is unfavorable. Rather, affected individuals require lifelong drug treatment to avoid triggering acute health conditions. In addition, secondary diseases are to be expected. Patients with paraproteinemia are often more susceptible to infections. The organism as a whole is weakened and can no longer react adequately to pathogens as usual. In the case of a very unfavorable development, the patient develops a tumor. As a result, there is a potential threat to the expected life expectancy and the ability to cope with everyday life is severely limited. Cancer therapy is necessary to achieve relief from the existing symptoms. Despite all efforts, in most patients the tumor disease ends in premature death. When making a prognosis, it must also be taken into account that psychological sequelae can occur due to the emotional stress caused by the general state of health. These also lead overall to a difficult further development and can be very protracted. In most cases, this also leads to further deterioration of physical health.
Prevention
Concrete measures for the prevention of paraproteinemia are not known according to the current state of medical research. For this reason, an appropriate specialist should be consulted at the slightest symptoms or signs of the disease. This is because the sooner the diagnosis is made, the more favorable the prognosis of paraproteinemia will be in some cases.
Follow-up
In most cases of paraproteinemia, the measures of a follow-up are very limited.Ideally, the person affected should consult a doctor at an early stage so that other complications and complaints do not arise for the person affected. The earlier a doctor is consulted, the better the further course of the disease usually is. Most patients are dependent on the removal of the tumor. After surgery, bed rest should be observed. Effort or stressful and physical activities should be refrained from in order not to put unnecessary strain on the body. Likewise, regular checks and examinations should also be carried out by a doctor in order to quickly detect and remove further tumors. Not infrequently, the psychological support of one’s own family is also very important, whereby especially loving and intensive conversations can have a positive effect on the further course of the disease. The further course of paraproteinemia strongly depends on the time of diagnosis, so that a general prediction is usually not possible. Possibly, this disease also limits the life expectancy of the affected person.
What you can do yourself
With this diagnosis, the patient must be thoroughly examined to determine what is underlying the paraproteinemia. This underlying condition may be a tumor and must be treated accordingly. The follow-up examinations due in the further course of the disease should also be observed, and any additional physical complaints should be taken seriously. Patients suffering from paraproteinemia usually feel tired and fatigued. In addition, there is an increased susceptibility to infections. To minimize these complaints, an appropriate lifestyle is helpful. This lifestyle is made up of various components. One 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 flush toxins out of the body, it is also advisable to drink plenty of fluids. Still mineral water, teas and thin juice spritzers have proven to be effective here. According to recent research, a healthy diet also has a positive influence on any depression that may exist. Since paraproteinemia is a very stressful diagnosis, additional therapeutic adjunctive therapy may be indicated. A paraproteinemia patient also benefits from a regular sleep and wake rhythm. They should avoid alcohol and nicotine and instead engage in regular exercise. Long walks in the woods or bike rides in the fresh air have proven effective here.
