Dysproteinemia

Patients with a Dysproteinemia suffer from a congenital or acquired disproportion in blood proteins. Since these proteins are produced in the liver, liver damage is behind the phenomenon in many cases. Treatment is done depending on the primary cause.

What is dysproteinemia?

The Greek prefix "Dys-" literally means something like "disturbance" or "malfunction". "Ämie" means "in the blood" in German. The compound word dysproteinemia is used in medical jargon for a disorder of the blood composition. Patients with dysproteinemia have abnormally composed protein levels in their blood.

The disorder results in a plasma protein disparity. The physician understands plasma proteins to be the cellular elements of the blood: i.e. the protein building blocks. Instead of a qualitative mismatch, dysproteinemia has a quantitative mismatch. The disorder essentially relates to the so-called albumins and globulins.

The globulins are storage proteins. Albumin is the only blood protein that is not counted among the globulins, but like globulins it is a globular protein. Dysproteinemia can be congenital or acquired. The phenomenon is usually the symptom of a general disease and does not correspond to an isolated phenomenon.

causes

The most common cause of dysproteinemia is the framework of a larger syndrome. The α₁-1-antitrypsin deficiency is one of the most important congenital causes. In addition, the congenital dysproteinemia occurs as defect dysproteinemia, analbuminemia or atransferrinaemia. Most congenital imbalances result from genetic mutations, which are often hereditary.

Acquired dysproteinemia can be caused by a wide variety of diseases. Many of them affect the main place where blood proteins are produced: the liver. In addition to causative liver diseases, so-called paraneoplastic syndromes can also provide the larger framework for dysproteinemia. In addition, different types of inflammation can cause blood protein mismatches.

The immune system is the primary trigger here. Acquired dysproteinemia also occurs when plasma protein is lost in the context of nephrotic syndrome. The antibody deficiency syndrome can also manifest itself through a disproportionate blood protein. The diseases mentioned are only to be understood as examples. Overall, the phenomenon can be symptomatic of numerous other diseases.

Symptoms, ailments & signs

Symptoms in patients with dysproteinemia differ depending on the primary cause. The congenital form, for example, is most often based on a metabolic α₁-1-antitrypsin deficiency. Most of the patients with this metabolic disease have a chronic, active liver inflammation.

This hepatitis can be painful and leads to extreme liver dysfunction which eventually causes dysproteinemia. In contrast, patients with congenital atransferrinaemia suffer from iron deposits in various organs. Depending on which organs are affected, there are organ-specific functional disorders in addition to dysproteinemia, such as renal insufficiency.

If the disproportion of blood proteins in the acquired form is present within the larger framework of a paraneoplastic syndrome, hormonally active tumors are the main symptom. By secreting hormones, these tumors simulate an endocrinological disease that can cause symptoms in various organs.

diagnosis

The diagnosis of dysproteinemia is made using serum electrophoresis. In this process, the blood proteins are separated and then quantified. Usually, the primary cause of dysproteinemia is diagnosed well before the symptom itself is diagnosed.

Only in rare individual cases is the diagnosis of dysproteinemia followed by a detailed diagnosis and research into the causes of the imbalance. The prognosis for patients with dysproteinemia depends on the primary cause. For example, patients with liver cirrhosis have a rather poor prognosis.

Complications

The complications and symptoms of dysproteinemia depend heavily on the cause of the symptom. As a rule, however, there is damage to the liver, which can have different effects on the patient's health. Most of those affected suffer from a metabolic disease.

This can manifest itself in the form of stomach pain, diarrhea, or gas. This often results in inflammation in the liver. Because of the liver problems, iron is deposited in various organs, which can ultimately lead to kidney damage. In the case of kidney failure, dialysis is then necessary to keep the patient alive. In the worst case scenario, cancer can develop.

The treatment is causal and primarily targets the underlying disease. In many cases, the person concerned has to avoid certain foods in order to stop glossing over the liver. Serious cases require surgical intervention.

If tumors occur due to dysproteinemia, they are also surgically removed or treated with the help of chemotherapy. Various complications can arise. It may be that a complete treatment of the dysproteinemia is not possible, so that it comes to a shortened life expectancy.

When should you go to the doctor?

Liver inflammation and symptoms of kidney failure may be caused by dysproteinemia. A visit to the doctor is recommended if the symptoms persist longer than usual or if they increase in intensity. In the event of complications, emergency medical help should always be requested. Depending on how early the disease is recognized, the affected person must then spend a few days or weeks in the hospital. By regularly consulting the responsible doctor, the appropriate treatment can be initiated quickly in the event of acute symptoms.

Acquired dysproteinemia occurs particularly in connection with liver diseases and so-called paraneoplastic syndromes. Inflammation as well as diseases such as nephrotic syndrome or antibody deficiency syndrome can lead to a disproportionate blood protein.

If these risk factors apply to you, you should definitely consult a doctor about the typical symptoms of dysproteinemia. For further examinations, depending on the symptoms, the hepatologist, the nephrologist or a specialist in internal medicine can be called in. In addition, a nutritionist should be consulted who will help with the change in diet.

Doctors & therapists in your area

Treatment & Therapy

As far as possible, a causal treatment of the primary disease is used in dysproteinemia. Symptomatic therapy would only try to correct the imbalance, but not eliminate the cause and thus achieve no real cure. In cirrhosis of the liver or other diseases with severe damage to the liver tissue, the standard therapy mainly includes supportive steps that are intended to prevent the disease from progressing.

From now on, patients must avoid all foods and consumables that are toxic to the liver or otherwise burden the liver. The causal cure for patients with liver cirrhosis is only possible through an organ transplant. Liver transplantation is also the final treatment option for patients with congenital dysproteinemia associated with alpha-1 antitrypsin deficiency.

Nevertheless, these patients initially receive substitutions for the deficient substance in order to keep the secondary diseases under control. Patients with paraneoplastic syndrome are treated invasively. During an operation, the hormone-producing tumor is removed as completely as possible.

Depending on the degree of malignancy, there is also radiation or chemotherapy. Immunoelectrophoresis has been available for some time for the symptomatic treatment of dysproteinemia. This procedure removes excess blood cells from the patient's blood.

The "cleaned" blood is returned to the patient in a similar way to dialysis. The procedure is not suitable for dysproteinemia of any cause. Immunoelectrophoresis can only be used for symptomatic treatment if the disproportion exists in the majority of certain blood proteins.

Outlook & forecast

The prognosis for dysproteinemia depends on the underlying cause and the severity of the disease. If there is no liver damage, there is a good chance that it will be cured. The imbalance in the protein balance is treated and treated. If the underlying disease is cured, the concentration of protein is then in a natural balance. The dysproteinemia is also considered cured in this case.

If there has already been tissue damage to the liver, the prognosis changes. The liver damage is irreparable and cannot be regenerated. In severe cases, there is a risk of organ failure. This is a life-threatening condition for the patient. If the patient suffers from a tumor, the prognosis is equally unfavorable. Surgical intervention and subsequent cancer therapy are carried out. The chances of recovery depend on the size of the tumor and the success of the therapy after tumor removal. In most cases, consequential damage will remain, but the patient's life can be saved.

If the patient has too many blood cells in his body, the blood is cleaned up. In this way, the dysproteinemia is cured. In the course of life, the blood may be cleaned again, as this procedure provides temporary relief from the symptoms and no cure.

prevention

Dysproteinemia can be the symptom of a number of diseases. If you want to prevent the disproportion of blood proteins, you would have to prevent a variety of different diseases. Since many of them are genetic, there are no hundred percent successful preventive measures available.

In principle, careful handling of substances that are toxic to the liver is recommended. The distance from alcohol can be interpreted in the broadest sense as one of dozens of preventive steps in the context of dysproteinemia.

Aftercare

In the case of dysproteinemia, in most cases there are no follow-up measures or options available to the person affected. First and foremost, a comprehensive diagnosis and examination should be carried out with this disease so that there are no further complications and no further worsening of the symptoms. The earlier the dysproteinemia is detected, the better the further course of this disease is usually.

With correct and, above all, early treatment of the disease, there is also no reduced life expectancy for the person affected. The treatment of this disease is always based on the underlying disease, so that it should be treated and, if possible, avoided. The further course of the dysproteinemia also depends very much on the underlying disease, so that no general prediction can be made about it.

If a tumor is responsible for the dysproteinemia, regular examinations are recommended even after removal in order to detect other tumors at an early stage. Supporting and caring for family or friends also has a very positive effect on the course of the disease and, above all, can prevent psychological upsets.

You can do that yourself

In the case of dysproteinemia, the physiological, i.e. normal, composition of the protein bodies in the blood is disturbed. The causes are very different and therefore dysproteinemia is only a symptom of very different disease processes. So if you wanted to prevent the disproportionate composition of blood proteins, you would have to prevent a whole range of different diseases.

This cannot be implemented in everyday life, especially since many of the dysproteinemias known today are clearly genetic. However, anyone who knows that they have one of the various forms of dysproteinemia should do everything possible to prevent the disease from worsening further.

In everyday life, this means in particular always careful and thoughtful handling of all substances and substances that are toxic to the liver. In certain professions, the use of paints, varnishes or solvents should definitely be avoided. However, the most important avoidable substance that is toxic to the liver in everyday life is alcohol.

In the interests of self-help in the presence of dysproteinemia, in the best case scenario a total abstinence from alcohol should be observed. Because even the consumption of the smallest amounts of alcohol has a much more toxic effect on patients with dysproteinemia than on those with healthy metabolism.