Monoclonal Gammopathy

Monoclonal gammopathy is a hematological disease. It is characterized by the overabundance of monoclonal antibodies. Monoclonal gammopathy affects the functioning of the immune system and can lead to anemia, hypercalcemia, ESR, hyper- or hypogammaglobulinemia and renal failure, among others.

Monoclonal Gammopathy

What is monoclonal gammopathy?

Monoclonal gammopathy is a hematological disease that affects the immune system. The defining feature of the clinical picture is the increased concentration of monoclonal antibodies that occur in the so-called gamma fraction of proteins in the blood serum. See nonprofitdictionary for Short Rib Polydactyly Syndrome (abbreviated as SRPS).

Antibodies are substances in the human body that help the immune system to recognize and then fight pathogens and foreign bodies. The addition “monoclonal” refers to the origin of these specific antibodies: The human body produces this type of antibody using a cell clone. All cell clones of an individual originate from one and the same cell, a so-called B lymphocyte.

Causes

Monoclonal gammopathy can be due to various causes, all of which 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 itself may be due to other diseases such as Waldenstrom’s disease, MGUS, or plasmacytoma. AL amyloidosis manifests itself in deposits of proteins inside and outside of cells. The consequences are diverse functional organ disorders and anatomical changes, for example in the form of edema, hardening and other pathological phenomena.

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 gangrenosum, and other underlying conditions can also lead to monoclonal gammopathy.

Symptoms, Ailments & Signs

Certain symptoms are typical of monoclonal gammopathy; Conversely, the presence of one, several or even all signs does not necessarily have to be due to a monoclonal gammopathy: In any 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 the anemia, symptoms such as dizziness, weakness, concentration problems and many others can manifest themselves.

In the context of monoclonal gammopathy, hypercalcaemia can also occur, which is characterized by a pathologically increased calcium level in the blood. Possible symptoms of severe hypercalcemia include loss of appetite, vomiting, nausea, constipation, mental symptoms, muscle hypotonia, and others.

Abnormalities in the blood cell sedimentation speed or reaction (blood sedimentation) are also typical. In addition, monoclonal gammopathy can lead to hyper- or hypogammaglobulinemia, i.e. to 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 ability to recognize and defend itself against potentially harmful invaders. Furthermore, monoclonal gammopathy can result in renal insufficiency.

Diagnosis & course of disease

When making a diagnosis, doctors first start from the existing symptoms and check whether typical symptoms of monoclonal gammopathy are present. If there is a suspicion, targeted tests are possible. One of them is immunoelectrophoresis, which can detect the relevant antibodies in the blood serum.

Before that, serum protein electrophoresis determines the serum proteins present on a more general level. The course of monoclonal gammopathy can be very variable, since the underlying disease plays a significant role in the chances of successful treatment and also affects the overall severity of the disease.

Complications

This disease is primarily associated with 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 those affected, resulting in considerable restrictions in everyday life. It is not uncommon for sleep disorders 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 thus a loss of appetite. The muscles are broken down and kidney failure can occur. This is usually fatal if left untreated. As a result, the affected person is dependent on a donor kidney or on dialysis in order to continue to survive.

The disease is treated with radiation therapy or chemotherapy. Chemotherapy usually leads to various unpleasant side effects. The disease cannot be completely cured in every case, so that in some cases the life expectancy of those affected is reduced. In particular, damage to the internal organs is often irreversible and can no longer be treated directly.

When should you go to the doctor?

If health impairments such as dizziness, inner weakness, malaise or a decrease in mental and physical performance occur, a doctor should be consulted. If everyday requirements can no longer be met as usual or if there is a problem with concentration and attention, a doctor should be consulted. If you have flu-like symptoms such as vomiting, nausea, fatigue, exhaustion or an increased need for sleep, you should consult a doctor. If the symptoms persist for several days or if they increase in scope and intensity, we recommend that you seek medical advice.

A reduced amount of oxygen in the body or respiratory disorders should be reported to a doctor. There is a threat of a functional failure of the organs, which can lead to a life-threatening condition for the person concerned. A doctor should be consulted if there are problems with the digestive tract, constipation or a feeling of fullness. Changes in going to the toilet, a reduced amount of urine or discoloration of the urine must be examined and treated. A general feeling of illness or reduced well-being are further signs of an irregularity. If, in addition to the physical problems, the person concerned also experiences mental or emotional inconveniences, a doctor’s visit is also necessary. If you have persistent or increasing mood swings,

Treatment & Therapy

The treatment of monoclonal gammopathy depends on the cause, which is present in the individual case. The prospect of successful therapy can vary greatly and cannot be generalized. In the case of AL amyloidosis as a result of a plasmacytoma or multiple myeloma, a bone marrow transplant may be an option, with which doctors try to treat the plasmacytoma causally.

More common options are chemotherapy and local radiation therapy, as well as various drug treatment approaches. Chemotherapy is also often an option for B-cell lymphoma. Successful treatment of AL amyloidosis can improve not only the monoclonal gammopathy; the disorders and changes in the affected organs such as the kidneys, heart, liver or intestines may also 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 an option. The nettle rash that also occurs with this syndrome is often difficult to treat; Options include PUVA therapy and non-steroidal anti -inflammatory drugs, with the latter also being used to treat joint and bone pain and fever episodes associated with Schnitzler syndrome.

Outlook & Forecast

The prognosis of monoclonal gammopathy depends on the form and stage of the disease. igM-MGUS offers a relatively poor prognosis, depending on any risk factors. All forms can develop into a serious illness over the years, which can sometimes be fatal for the patient. For example, malignant tumors can appear in different parts of the body. The likelihood of serious complications increases with age. Life expectancy is slightly lower than that of healthy people. There is a risk that the condition will develop into a chronic condition that will severely limit the patient’s daily life.

The prognosis is made by the responsible oncologist or another specialist with regard to factors such as the symptoms, the form of the disease and the age of the patient. Regardless of the prognosis, however, many sufferers can lead a relatively symptom-free life. The physical limitations usually develop slowly and do not significantly impair the patient’s quality of life. Only in the case of malignant diseases can a rapid progression with a progressive decrease in well-being be expected. The malignant form of monoclonal gammopathy can be fatal.

Prevention

Specific prevention of monoclonal gammopathy is not possible. Early detection and treatment of the causative disease can possibly prevent severe courses and creates the basis for early therapy of monoclonal gammopathy.

Aftercare

Monoclonal gammopathies are usually long-term diseases that often require intensive follow-up care. Which aftercare measures have to be taken depends on the type and course of the individual disease. In many cases, these are low-grade malignant lymphomas that are classified as non-Hodgkin lymphomas.

Radiation and chemotherapy often do not lead to complete healing of these lymphomas. However, the symptoms can be significantly alleviated and the quality of life of those affected can be improved. Follow-up care includes regular examinations that record the patient’s condition and at the same time determine further treatment.

In the event of a deterioration in the state of 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, since recurrences can still occur even after several years. However, monoclonal gammopathy often requires lifelong follow-up care.

This is particularly true when the disease can no longer be cured using conventional treatment methods. In these cases, follow-up care is palliative. 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 often reduced.

You can do that yourself

The clinical picture of a monoclonal gammopathy is complex and diverse. For this reason, dealing with the disease in everyday life depends heavily on its cause, the therapy methods chosen and the symptoms present. Patients whose symptoms are caused by a malignant disease can turn to cancer self-help groups and organizations. Their relatives can also find help there in dealing with the sick person.

Since the treatment of the underlying disease can put a lot of strain on the organism, it is advisable to strengthen the immune system. A balanced, vitamin-rich diet plays an important role here. Additional stress, for example due to a heavy workload, must be avoided at all costs. In some cases, despite a finding of monoclonal gammopathy, there are no symptoms of a disease. Those affected can also preventively strengthen their immune system. Physical exertion and mental stress should be avoided at least in the first year after the initial diagnosis.

Regular medical check-ups are important. As soon as those affected notice physical symptoms, a doctor should be consulted and, if necessary, a new blood count should be drawn up. Otherwise, alternative healing methods can also be helpful in monoclonal gammopathy and contribute to general physical relaxation. Even light exercise can help those affected.