Monoclonal gammopathy is a hematologic disorder. It is characterized by the excessive occurrence of monoclonal antibodies. Monoclonal gammopathy affects the functioning of the immune system and can lead to anemia, hypercalcemia, blood sedimentation, hyper- or hypogammaglobulinemia, and renal insufficiency, among other symptoms.
What is monoclonal gammopathy?
Monoclonal gammopathy is a hematologic disorder that has effects from the immune system. The defining feature of the clinical picture is the increased concentration of monoclonal antibodies found in the so-called gamma fraction of proteins in blood serum. Antibodies are substances in the human body that help the immune system to recognize and subsequently fight pathogens and foreign bodies. The term “monoclonal” refers to the origin of these specific antibodies: the human body produces this type of antibody with the help of a cell clone. All cell clones of an individual are derived from one and the same cell, a so-called B lymphocyte.
Causes
Monoclonal gammopathy may be due to several causes that have in common that the crucial cell clone is altered. The affected cell clone multiplies uncontrollably and in this way causes various physical disorders. One disease that can lead to monoclonal gammopathy is AL amyloidosis, which in turn may be due to other diseases such as Waldenström’s disease, MGUS, or plasmacytoma. AL amyloidosis manifests as deposits of proteins inside and outside cells. The result is a variety of functional organ disorders and anatomical changes, for example in the form of edema, induration and other pathological manifestations. Schnitzler syndrome can also lead to monoclonal gammopathy. Schnitzler syndrome is a rare disease in which monoclonal gammopathy is associated with chronic hives (urticaria) and joint pain. B-cell lymphoma, non-Hodgkin’s lymphoma, pyoderma gangraenosum, and other underlying diseases can also lead to monoclonal gammopathy.
Symptoms, complaints, and signs
Certain symptoms are typical of monoclonal gammopathy; conversely, the presence of one, several, or even all signs is not necessarily due to monoclonal gammopathy: In each case, an individual and comprehensive diagnosis is required. In many cases, monoclonal gammopathy leads to anemia, often referred to as anemia. This is a deficiency of the red blood pigment hemoglobin, which plays a central role in the transport of oxygen. As a result of anemia, symptoms such as dizziness, feeling weak, difficulty concentrating, and many others may manifest. In addition, in the context of monoclonal gammopathy, hypercalcemia may occur, which is characterized by pathologically elevated calcium levels in the blood. Possible symptoms of severe hypercalcemia include loss of appetite, vomiting, nausea, constipation, psychological symptoms, muscle hypotonia, and others. Abnormalities in blood cell sedimentation rate or response (blood sedimentation) are also typical. In addition, monoclonal gammopathy may lead to hyper- or hypogammaglobulinemia, i.e., a significant increase or decrease in certain plasma proteins, namely gamma globulins. Gamma globulins are also part of the immune system and play a role in the body’s recognition and defense against potentially harmful invaders. Furthermore, monoclonal gammopathy can result in renal insufficiency.
Diagnosis and disease progression
When making a diagnosis, physicians first start from the presenting symptoms and check whether typical symptoms of monoclonal gammopathy are present. If there is a suspicion, targeted tests are possible. One of these is immunoelectrophoresis, which can detect the relevant antibodies in the blood serum. Prior to this, serum protein electrophoresis determines the serum proteins present at a more general level. The course of monoclonal gammopathy can vary widely, as the underlying disease plays a major role in the chances of successful treatment and also affects the severity of the clinical picture overall.
Complications
The primary complication of this disease is severe anemia.As a result, those affected suffer from reduced resilience and severe fatigue. Certain everyday activities or sporting activities are no longer possible for the affected person, resulting in considerable restrictions in everyday life. It is not uncommon for sleep problems and concentration disorders to occur. A feeling of weakness also occurs and can reduce the patient’s quality of life. It is not uncommon for patients to also suffer from nausea, vomiting and consequently a loss of appetite. Muscles are degraded and renal insufficiency may occur. Without treatment, this is usually fatal. The affected person is thus dependent on a donor kidney or dialysis in order to continue to survive. Treatment of the disease takes place with the help of radiation therapy or chemotherapy. Chemotherapy usually leads to various unpleasant side effects. The disease is not completely curable in every case, so that in some cases the life expectancy of the affected person is reduced. Damage to the internal organs in particular is often irreversible and can no longer be treated directly.
When should you see a doctor?
If health impairments such as dizziness, internal weakness, malaise, or a decrease in mental as well as physical performance occur, a physician should be consulted. If everyday requirements can no longer be met as usual or if there is a disturbance of concentration and attention, a visit to the doctor should be made. If flu-like symptoms such as vomiting, nausea, faintness, exhaustion or an increased need for sleep occur, a doctor should be consulted. If the symptoms persist over several days or increase in scope and intensity, medical clarification is recommended. A reduced amount of oxygen in the organism or disturbance of respiratory activity should be presented to a doctor. There is a risk of organ dysfunction, which can lead to a life-threatening condition of the affected person. In case of disorders of the digestive tract, constipation or a feeling of fullness, a doctor should be consulted. Changes in toileting, decreased urine output, or discoloration of the urine should be investigated and treated. A general feeling of illness or decreased well-being are other signs of irregularity. If, in addition to the physical problems, the affected person experiences mental or emotional discomfort, a visit to the doctor is also necessary. In case of persistent or increasing mood swings, abnormalities in behavior or a depressive appearance, the affected person needs medical help as well as medical care.
Treatment and therapy
Treatment of monoclonal gammopathy depends on the cause in each individual case. The prospect of successful therapy can vary widely and cannot be generalized. In cases of AL amyloidosis due to plasmacytoma or multiple myeloma, bone marrow transplantation may be considered, with which physicians attempt to treat the plasmacytoma causally. More common options include chemotherapy and local radiation therapy, as well as various drug treatment approaches. Chemotherapy is also often an option for B-cell lymphoma. If treatment of AL amyloidosis is successful, not only may the monoclonal gammopathy improve; it is also possible that the disorders and changes in affected organs such as the kidneys, heart, liver, or intestines may be reversible. However, this also depends on the individual case. If the monoclonal gammopathy is due to Schnitzler syndrome, treatment with interleukin-1 antagonists may be considered. The hives that also occur in this syndrome are often difficult to treat; options include PUVA therapy and nonsteroidal anti-inflammatory drugs, the latter of which may also be used for joint and bone pain and fevers associated with Schnitzler syndrome.
Outlook and prognosis
The prognosis of monoclonal gammopathy is based on the disease form and stage. igM-MGUS offers a relatively poor prognosis, depending on any risk factors. All forms can develop over a period of years into a severe disease that may be fatal to the patient. For example, malignant malignancies may occur at various sites in the body.The likelihood of serious complications increases with age. Life expectancy is somewhat lower than that of healthy people. There is a risk that the condition will develop into a chronic disease that significantly limits the patient’s daily life. The prognosis is made by the oncologist in charge or another specialist with regard to factors such as the symptom picture, the form of the disease and the patient’s age. Regardless of the prognosis, however, many patients can lead a relatively symptom-free life. The physical limitations usually develop slowly and do not significantly restrict the patient’s quality of life. Only in the case of malignant diseases is a rapid course with a progressive decline in well-being to be expected. The malignant form of monoclonal gammopathy can be fatal.
Prevention
Specific prevention of monoclonal gammopathy is not possible. Early recognition and treatment of the causative disease can potentially prevent severe courses and lays the foundation for early therapy of monoclonal gammopathy.
Follow-up
Monoclonal gammopathies are usually long-lasting diseases that often require intensive follow-up. The follow-up measures that need to be taken depend on the nature and course of the individual disease. Many cases are low-malignant lymphomas, which are classified as non-Hodgkin’s lymphomas. Radiation and chemotherapies still often do not lead to a complete cure of these lymphomas. However, the symptoms can be significantly alleviated, thereby improving the quality of life of those affected. Follow-up care includes regular examinations to assess the patient’s condition and at the same time determine further treatment. In the event of any deterioration in the patient’s health, it is also possible to react quickly. Even after successful treatment of the disease, further visits to the doctor should be made over a longer period of time, as recurrences can still occur even after several years. Often, however, lifelong follow-up is necessary in the case of monoclonal gammopathy. This is especially true if the disease is no longer curable after conventional treatment methods. In these cases, follow-up care has a palliative character. Due to the protracted course of the disease, those affected also very often need psychological counseling, among other things to prevent depression. For many patients, psychotherapy can therefore help to significantly improve their quality of life. Fears about the course of the disease and possible death are also frequently reduced in the process.
What you can do yourself
The clinical picture of monoclonal gammopathy is complex and varied. For this reason, dealing with the disease on a day-to-day basis depends greatly on its cause, the therapeutic methods chosen, and the symptoms present. Affected persons for whom a malignant disease is the cause of the symptoms can turn to self-help groups and organizations for cancer. There, their relatives can also find help in dealing with the sufferer. Since the treatment of the underlying disease can put a great strain on the organism, it is advisable to strengthen the immune system. A balanced diet rich in vitamins plays a particularly important role here. Additional stress, for example due to a heavy workload, should be avoided at all costs. In some cases, there are no symptoms of a disease despite the presence of a monoclonal gammopathy. These patients can also strengthen their defenses as a preventive measure. At least in the first year after the initial diagnosis, physical exertion and psychological stress should be avoided. Regular medical check-ups are important. As soon as affected persons notice physical complaints, a doctor should be consulted and, if necessary, a new blood count should be taken. Otherwise, in the case of monoclonal gammopathy, alternative healing methods can also be helpful and contribute to a general physical relaxation. Light exercise can also help those affected.
