POEMS syndrome is a rare variation of multiple myeloma with associated paraneoplasia. Elevated levels of vascular endothelial growth factors (VEGF) can be detected in almost all patients.
What is POEMS Syndrome?
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. It is not yet known how the POEMS syndrome develops.
The syndrome occurs most frequently between the ages of 50 and 60. It is often confused with chronic inflammatory demyelinating polyneuropathy. A dangerous misdiagnosis, because the therapy of the chronic inflammatory demyelinating polyneuropathy is absolutely ineffective in POEMS syndrome. Treatment of POEMS syndrome is with high-dose chemotherapy and stem cell transplantation. See AbbreviationFinder for abbreviations related to POEMS.
The origin of the multisystem 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 the POEMS syndrome, the increased VEGF serum level seems to be reflected in an increased activity of the plasma cells. In higher amounts, VEGF appears to increase vascular permeability. This leads to fluid retention in the tissue, which leads to nerve damage.
Symptoms, Ailments & Signs
POEMS syndrome is a multisystem disease. Accordingly, the disease has many faces. Not every patient shows every symptom. Many patients complain of symptoms that can be traced back to damage to the peripheral nerves. This damage is also known as peripheral polyneuropathy. There are sensory disturbances in the extremities (acroparesthesia), abnormal sensations and motor failures.
Later, the nerves become sensitive to pressure. This can be noticed, for example, by calf pressure pain. Nutritional disorders in the form of skin damage can also result from damage to the autonomic nerves. The syndrome also manifests itself in all patients as a monoclonal plasma cell disease.
This changes the composition of the proteins in the blood plasma. Usually an immunoglobulin multiplies pathologically. Multiple myeloma is one of the most common monoclonal plasma cell diseases associated with POEMS syndrome. In most cases it is an IgG plasmacytoma. The plasma cells in the bone marrow multiply and produce complete or incomplete type G immunoglobulins.
Due to the destructive spread of the cell clones in the bones, the other stem cells involved in blood formation are displaced. This leads to anemia with symptoms such as tiredness, susceptibility to infections and problems concentrating. Since fewer platelets are also produced, patients have an increased tendency to bleed. The immunoglobulins also inhibit the activity of the osteoblasts in the bones. There is a dissolution of the bone with a subsequent increase in the level of calcium in the blood.
Osteolysis makes itself felt in patients in the form of back pain or shoulder pain. Hypercalcemia can damage the kidneys. Kidney failure 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 may also develop as the disease progresses. In endocrinopathy, hormone production, hormone regulation or hormone effects are disrupted. Known endocrinopathies are Graves ‘ disease, type 2 diabetes mellitus, Cushing’s syndrome, hypothyroidism and hyperthyroidism.
In many patients, the disease initially manifests itself 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
For diagnosis, the symptoms of the POEMS syndrome can be divided into mandatory criteria, main criteria and secondary criteria. In order for the diagnosis of POEMS syndrome to be made, the two mandatory criteria must be met. In addition, a main and a secondary criterion must be present. The two mandatory criteria are peripheral polyneuropathy and monoclonal plasma cell disease.
Major criteria include elevated serum VEGF levels, Castleman’s disease, and sclerotic bone lesions. Additional criteria include organomegaly, skin changes, erythrocytosis, thrombocytosis, endocrinopathy, papilledema and excess volume outside the vessels.
The POEMS syndrome does not always lead to complications or symptoms. In many cases, those affected can lead a symptom-free life. However, paralysis and other sensory disturbances occur in most cases due to the syndrome. The patient’s everyday life may also be restricted, so that those affected are dependent on the help of other people in their lives.
Pain can also occur in the calves. The nerves themselves are irreversibly damaged and can no longer be restored. Patients with POEMS syndrome also suffer from concentration disorders and fatigue. 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, it usually leads to kidney problems and, in the worst case, to kidney failure. The POEMS syndrome cannot be treated causally. 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 sensory disturbances should consult a doctor. If there are disorders of the musculoskeletal system, abnormal sensations or unsteady gait, a doctor is needed. A doctor is needed in the event of pressure pain, complications with locomotion or a restriction in mobility. If the symptoms persist for a long time or if they continue to spread, the person concerned needs help. A susceptibility to infection, pain in the back or shoulders indicate health problems that need to be examined and treated.
An increased bleeding tendency should be presented to a doctor. If existing bleeding cannot be stopped, medical treatment is necessary. Tiredness, exhaustion and a drop in physical and mental performance levels should be discussed with a doctor.
Disorders of concentration and attention, reduced well-being and a decrease in quality of life should be discussed with a doctor. If the person concerned suffers from problems with urination or pain in the kidney area, a comprehensive examination is necessary to clarify the cause. Blood in the urine or a general feeling of being unwell is considered a concern. If the person concerned suffers from enlarged lymph and a feeling of illness for a longer period of time, it is advisable to consult a doctor.
Treatment & Therapy
The treatment of the POEMS syndrome is based on the guidelines for the therapy of plasmacytoma. This is multimodal and interdisciplinary. The disease cannot be cured, but it can be treated. The goals of therapy are to reduce symptoms and increase survival time. In addition, possible complications should be avoided. With 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 is intended to reduce the concentration of immunoglobulins in the blood and 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. Pamidronate is infused monthly for this. Hypercalcaemia is also treated with medication.
Outlook & Forecast
The prognosis for patients with POEMS syndrome is difficult. In many cases, the signs and symptoms present are misinterpreted as chronic inflammatory demyelinating polyneuropathy. The problem is that the treatment is ineffective. There is no symptomatic treatment of the syndrome that is actually present. This can have dramatic consequences for those affected.
The paraneoplastic POEMS syndrome definitely reduces the survival time. The leading symptoms are polyneuropathy, organomegaly, endocrinopathy, M gradient and skin changes. However, these symptoms usually appear more frequently in later life. In this respect, the limitation of lifespan in POEMS syndrome does not always have to be regarded as serious.
Since the causes of POEMS syndrome are unknown, targeted prevention is often not possible at a younger age. Multimodal therapies are mostly used to treat POEMS syndrome. These aim to improve the quality of life. The only therapeutic measures currently available are the administration of high-dose chemotherapeutic agents, or stem cell transplantation as the gold standard for younger patients.
If left untreated, the prognosis of POEMS syndrome worsens. Kidney problems and kidney failure 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 prevent complications and increase survival time.
So far it has not been clarified how and why the POEMS syndrome develops. There are therefore no effective preventive measures.
In most cases, patients with POEMS syndrome have no special aftercare options, since 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 sooner a doctor is consulted, the better the further course of this disease. As a rule, those affected by POEMS syndrome are dependent on taking various medications that can permanently relieve 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, always consult a doctor first. In many cases, patients are also dependent on the help and care of their own families due to the disease. Loving and intensive conversations also have a positive effect on the further course of the disease and can prevent depression or other mental illnesses. In some cases, however, POEMS syndrome reduces the life expectancy of the affected person.
You can do that yourself
This disease has many faces, so it may have taken a long time to diagnose it. This is just as stressful for many patients as the therapies that follow or the fact that they are dependent on the help of others. Therefore, it is definitely advisable to seek psychotherapeutic support during this time.
Joining a self-help group is also perceived as a relief by many patients. 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 Deutschland” website (www.myelom-deutschland.de/ selbsthilfegruppen/).
Since POEMS syndrome is associated with susceptibility to infections, those affected can help themselves by strengthening their immune system with a healthy lifestyle. On the one hand, this means that the patients do not smoke and drink as little alcohol as possible so as not to damage their bodies further. 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 located in the intestine, the administration of probiotics is also recommended. These are dietary supplements that contain live microorganisms. They multiply in the intestines and are said to contribute to the maintenance of the immune system there.