POEMS syndrome

The POEMS syndrome is a rare variation of multiple myeloma with accompanying paraneoplasia. In almost all patients, increased values ​​of the vascular endothelial growth factor (VEGF) can be detected.

What is POEMS Syndrome?

The POEMS syndrome is a paraneoplastic disease. The acronym POEMS stands for the English names of the symptoms polyneuropathy, endocrinopathy, M-gradient, skin changes and organomegaly. How the POEMS syndrome develops is not yet known.

The syndrome most often occurs between the ages of 50 and 60. It is often confused with chronic inflammatory demyelinating polyneuropathy. A dangerous misdiagnosis, because the therapy of chronic inflammatory demyelinating polyneuropathy is absolutely ineffective in POEMS syndrome. The POEMS syndrome is treated with high-dose chemotherapy and stem cell transplantation.

causes

The origin of the multi-system disease is still unclear. However, the cytokine VEGF seems to play a crucial role in the pathogenesis. In patients with POEMS syndrome, the VEGF serum level is significantly increased. It also correlates with disease activity. This means that the higher the VEGF level, the more pronounced the symptoms.

VEGF is an endothelial growth factor that is normally required for the formation of new vessels and new endothelial cells. In POEMS syndrome, the increased VEGF serum level appears to be a result of increased activity of the plasma cells. In higher amounts, VEGF appears to increase the permeability of the vessels. This leads to fluid retention in the tissue, which leads to nerve damage.

Symptoms, ailments & signs

POEMS syndrome is a multi-system disease. Accordingly, the disease has many faces. Not every patient shows every symptom. Many patients complain of symptoms that can be attributed to damage to the peripheral nerves. This damage is also known as peripheral polyneuropathy. Sensitivity disorders in the extremities (acroparaesthesia), abnormal sensations and motor failures occur.

Later, the nerves are also sensitive to pressure. This can be noticeable, for example, through pain in the calf pressure. Nutritional disorders in the form of skin damage can also result from damage to the vegetative nerves. The syndrome also manifests itself in all patients as monoclonal plasma cell disease.

This changes the composition of the proteins in the blood plasma. An immunoglobulin usually reproduces pathologically. One of the most common monoclonal plasma cell diseases that occur in the context of POEMS syndrome is multiple myeloma. In most cases it is an IgG plasmacytoma. The plasma cells in the bone marrow multiply and produce complete or incomplete immunoglobulins of type G.

The destructive spread of the cell clones in the bones displaces the other stem cells of the blood formation. This results in anemia with symptoms such as tiredness, susceptibility to infections and difficulty concentrating. Since fewer platelets are produced, patients have an increased tendency to bleed. The immunoglobulins also inhibit the activity of the osteoblasts in the bones. The bone dissolves and the calcium level in the blood increases.

Osteolysis makes itself felt in the patient in the form of back pain or shoulder pain. The hypercalcemia can damage the kidney. Renal insufficiency may even develop. Many patients with POEMS syndrome also have Castleman's disease. Here one or more lymph nodes are enlarged due to hyperplasia.

Endocrinopathy can also develop as the disease progresses. In the case of endocrinopathy, the hormone production, the hormone regulation or the hormone effect are disturbed. Well-known endocrinopathies are Graves' disease, type 2 diabetes mellitus, Cushing's syndrome, hypothyroidism and hyperthyroidism.

In many patients, the disease first shows up in the form of skin changes. These vary from patient to patient and have neither a clearly defined appearance nor a preferred location.

Diagnosis & course of disease

To make a diagnosis, the symptoms of POEMS syndrome can be divided into mandatory, main and secondary criteria. In order for the diagnosis of POEMS syndrome to be made, the two mandatory criteria must be met. In addition, there must be a main and a secondary criterion. The two mandatory criteria are peripheral polyneuropathy and monoclonal plasma cell disease.

The main criteria include increased VEGF serum levels, Castleman's disease, and sclerotic bone lesions. Additional criteria include organomegaly, skin changes, erythrocytosis, thrombocytosis, endocrinopathy as well as congestive papilla and excess volume outside the vessels.

Complications

The POEMS syndrome does not have to lead to complications or symptoms in every case. In many cases, those affected can lead a symptom-free life. However, in most cases, the syndrome leads to paralysis and other disorders of sensitivity. It may also affect the patient's everyday life, so that those affected depend on the help of other people in their lives.

You may also experience pain in the calves. The nerves themselves are irreversibly damaged and cannot be restored. The patients also suffer from concentration disorders and fatigue due to the POEMS syndrome. Due to the weakened immune system, patients are often susceptible to infections and various diseases. Back pain can also occur with this disease.

If the POEMS syndrome is not treated, kidney problems and, in the worst case, kidney failure occur. The cause of the POEMS syndrome cannot be treated. For this reason, only the symptoms are treated. The life expectancy of those affected is usually reduced by the POEMS syndrome.

When should you go to the doctor?

People who suffer from ailments of the extremities and have sensitivity disorders should consult a doctor. A doctor is required if there are disorders of the musculoskeletal system, abnormal sensations or unsteady gait. A doctor is needed in the event of tenderness, complications in locomotion or restricted mobility. If the symptoms persist for a long time or if they spread further, the person affected needs help. A susceptibility to infection, pain in the back or shoulders indicate health impairments that must be examined and treated.

An increased bleeding tendency should be presented to a doctor. If existing bleeding cannot be stopped, it is necessary to seek medical treatment. Fatigue, exhaustion and a drop in physical and mental performance should be discussed with a doctor.

Disturbances in concentration and attention, decreased well-being and a decrease in quality of life should be discussed with a doctor. If the person concerned suffers from urination disorders or pain in the kidney area, an extensive examination is necessary to clarify the cause. Blood in the urine or a general feeling of malaise is considered to be a concern. If the person concerned suffers from enlarged lymph and a feeling of illness over a long period of time, it is advisable to consult a doctor.

Therapy & Treatment

The treatment of the POEMS syndrome is based on the guidelines for the therapy of plasmacytoma. This is multimodal and interdisciplinary. The disease is not curable, but treatable. The goals of therapy are symptomatic relief and survival time. In addition, possible complications should be prevented. For a plasmacytoma from stage II and in patients over 65 years of age, chemotherapy is carried out according to the Alexanian scheme with melphalan and prednisone.

This should reduce the immunoglobulin concentration in the blood and reduce the plasma cells in the bone marrow by 50 percent. With this partial normalization of the laboratory values, the patient's condition also improves. Alternatively, thalidomide or bortezomib can also be used as chemotherapeutic agents. In younger patients, the gold standard is autogenous stem cell transplantation from the bone marrow.

This must be combined with high-dose myeloablative chemotherapy. Allogeneic stem cell transplantation from histocompatible bone marrow is gentler. However, the recurrence rate is higher here. Patients also receive bisphosphonates to reduce bone dissolution. For this, pamidronate is infused monthly. Hypercalcemia is also treated with medication.

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Outlook & forecast

The prognosis for patients with POEMS syndrome is difficult. In many cases the present signs and symptoms are misinterpreted as chronic inflammatory demyelinating polyneuropathy. The problem is that the treatment remains ineffective. There is no symptomatic treatment of the actual syndrome. This can have dramatic consequences for those affected.

The paraneoplastic POEMS syndrome definitely reduces survival time. The main symptoms are polyneuropathy, organomegaly, endocrinopathy, M-gradient and skin changes. However, these symptoms usually come to light more frequently in later life. In this respect, the limitation of the lifetime in POEMS syndrome does not always have to be viewed as serious.

Since the causes for the development of POEMS syndrome are unknown, targeted prevention is often not possible in younger years. Multimodal therapies are mostly used to treat POEMS syndrome. The aim of these is to improve the quality of life. The only therapeutic measures currently available are the administration of high-dose chemotherapeutic agents or, for younger sufferers, stem cell transplantation as the gold standard.

If left untreated, the prognosis for POEMS syndrome worsens. Kidney problems and renal insufficiency can occur. These can only be remedied with dialysis or an organ transplant. Those affected may also have an increased tendency to bleed or a weakened immune system. Treatment aims to avoid complications and to extend survival time.

prevention

So far it is not clear how and why the POEMS syndrome develops. There are therefore no effective preventive measures.

Aftercare

In most cases, the patient with POEMS syndrome does not have any special follow-up options, as this disease should first and foremost be treated comprehensively and correctly by a doctor. Only with early detection and subsequent treatment of the disease can further complications be prevented.

The earlier a doctor is consulted, the better the further course of this disease is usually. As a rule, those affected with POEMS syndrome are dependent on the intake of various medications that can permanently alleviate the symptoms. It is always important to ensure that the medication is taken correctly and that the dosage is correct.

If you have any questions or are unclear, you should always consult a doctor first. In many cases, the disease also means that patients are dependent on the help and care of their own families. Loving and intensive conversations also have a positive effect on the further course of the disease and can prevent depression or other mental illnesses. However, in some cases, POEMS syndrome reduces the life expectancy of those affected.

You can do that yourself

This condition has many faces, so it may have taken a long time to make a diagnosis. This is just as stressful for many patients as the following therapies or the fact that they are dependent on the help of others. It is therefore definitely advisable to seek psychotherapeutic support during this time.

Many patients also find joining a self-help group a relief. Since the POEMS syndrome is a variant of multiple myeloma, a list of all self-help groups can be found on the Internet on the “Myelom Germany” website (www.myelom-deutschland.de/selbsthilfegruppen/).

Since POEMS syndrome is associated with susceptibility to infection, those affected can help themselves by strengthening their immune system with a healthy lifestyle. On the one hand, this means that patients do not smoke and drink as little alcohol as possible so as not to cause additional damage to their bodies. On the other hand, a healthy lifestyle also includes a diet that contains sufficient vitamins and minerals. A regulated sleep-wake cycle and plenty of exercise in the fresh air also support the immune system.

Because eighty percent of all immune cells are in the intestine, the administration of probiotics is also recommended. These are food supplements that contain live microorganisms. They multiply in the intestine and are supposed to help maintain the immune system there.