Definitions of Dysproteinemia
Patients with dysproteinemia suffer from a congenital or acquired disparity in blood proteins. Since these proteins are produced in the liver, in many cases there is liver damage behind the phenomenon. Treatment is based on the primary cause.
What is dysproteinemia?
According to Whicheverhealth, 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 disproportion between the plasma proteins. The medical professional 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 illness and does not correspond to an isolated phenomenon.
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, as analbuminemia or atransferrinemia. 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 causal 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 with plasma protein losses in the context of nephrotic syndrome. Antibody deficiency syndrome can also manifest itself as a disproportionate proportion of blood proteins. 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 innate form, for example, is most often based on a metabolic α₁-1-antitrypsin deficiency. Most patients with this metabolic disorder have a chronic, active liver inflammation.
This hepatitis can be painful and leads to extreme liver dysfunction which eventually leads to 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 kidney failure.
If the mismatch 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.
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 cause of the imbalance. The prognosis for patients with dysproteinemia depends on the primary cause. For example, patients with cirrhosis of the liver have a rather poor prognosis.
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 leads to 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 renal insufficiency, dialysis is then necessary to keep the patient alive. In the worst case, 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. In some cases, complete treatment of the dysproteinemia is not possible, so that life expectancy is shortened.
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 and diseases such as nephrotic syndrome or antibody deficiency syndrome can also 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.
Treatment & Therapy
As far as possible, a causal treatment of the primary disease is used in dysproteinemia. Symptomatic therapy would only try to compensate for 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 stress 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 due to alpha-1 antitrypsin deficiency.
Nonetheless, 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, radiation or chemotherapy is also carried out. Stands for the symptomatic treatment of dysproteinaemias for some time that immunoelectrophoresis available. 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 of 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 the tumor has been removed. In most cases, sequelae 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. 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.
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 multitude of different diseases. Since many of them are genetically determined, no one hundred percent successful preventive measures are available.
It is generally advisable to deal carefully with substances that are toxic to the liver. The distance from alcohol can broadly be interpreted as one of dozen preventive steps in the context of dysproteinemia.
In the case of dysproteinemia, in most cases there are no measures or options for follow-up care available to the person affected. First and foremost, a comprehensive diagnosis and examination should be carried out for 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 also.
With correct and, above all, early treatment of the disease, there is also no reduction in the life expectancy of the person affected. The treatment of this disease is always based on the underlying disease, so that it should be treated and also avoided as far as possible. 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 for this are very different and therefore dysproteinemia is only a symptom of very different disease processes. So if you wanted to prevent the imbalance of an incorrect composition of blood proteins, you would also 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 considered handling of all substances and substances that are toxic to the liver. In certain professions, the use of paints, varnishes or solvents should be avoided at all costs. 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 case 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.