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POEMS Syndrome Related Conditions Infosheet

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POEMS Syndrome Related Conditions Infosheet POEMS syndrome Related conditions Infosheet This Infosheet explains what POEMS syndrome is, how it is diagnosed and how it is treated and managed. What is POEMS syndrome? • Endocrinopathy – abnormal POEMS syndrome is a rare disorder function of endocrine (hormone- that can affect multiple systems producing) glands in the body. It is named after five • Monoclonal plasma cell common features of the syndrome, disorder – disorder involving described below: abnormal plasma cells (see over) Polyneuropathy – nerve • • Skin changes – certain skin damage to various peripheral changes are characteristic in nerves which control muscles POEMS syndrome, such as and sensation. Also known as hyperpigmentation (darkening peripheral neuropathy of an area of the skin) • Organomegaly – enlargement of organs, such as the liver, spleen or lymph nodes Infoline: 0800 980 3332 1 There are also a range of other What is a monoclonal features (“criteria”) that may occur plasma cell disorder? in POEMS syndrome. However, not A plasma cell disorder is the term every patient will have them. The used to describe a condition which features include: produces abnormal plasma cells. Plasma cells are a type of white • Abnormal (“sclerotic”) bone growth in certain parts of the blood cell that produce antibodies skeleton. They are typically (immunoglobulins). Antibodies painless but are usually visible on bind to substances in the body that X-rays and scans are recognised as foreign, such as bacteria and viruses (known as Swelling of the optic nerve, the • antigens), enabling other cells of main nerve in the eye (known as the immune system to destroy and papilloedema) remove them. Fluid build-up around the lungs, • In monoclonal plasma cell disorders, in the abdomen and/or the legs large numbers of identical plasma cells are produced. The cells • High red blood cell levels and/or high platelet levels in the blood produce an abnormal antibody known as paraprotein (also called Raised levels of a cytokine • monoclonal or M protein) which (chemical messenger) in has no useful function. In POEMS the body known as vascular syndrome, the abnormal plasma endothelial growth factor (VEGF) cells may be located in one or more (occurs in most POEMS patients) specific areas of the bone marrow (termed ‘plasmacytomas’), or may • Castleman disease (a type of lymph node disorder which be present throughout the bone causes a range of symptoms) marrow. The number of abnormal plasma cells in the bone marrow is POEMS syndrome is also known as generally small, and M protein levels osteosclerotic myeloma, Takatsuki are also generally low. syndrome and Crow-Fukase syndrome, however, these terms What causes POEMS are less commonly used. syndrome? The exact causes of POEMS syndrome are not well understood. 2 myeloma.org.uk The paraprotein produced by the syndrome. Peripheral neuropathy is abnormal plasma cells is on its own often the most debilitating feature not enough to explain the many of POEMS syndrome. features of POEMS syndrome. The first signs of peripheral Various cytokines are thought to neuropathy include numbness and play a role in causing damage to tingling in the hands and feet which the different tissues and organs progressively worsens over time. involved in the syndrome. This Pain, discomfort and weakness in includes the cytokine VEGF that the hands and feet are common is found in higher levels in POEMS features of POEMS syndrome syndrome patients. and weakness is often an early symptom of the syndrome. Who can develop POEMS syndrome? Other symptoms vary depending on the organ systems involved and can POEMS syndrome is very rare and include: the incidence is not fully known. It is thought that many cases • Weight loss remain undiagnosed because of its Diarrhoea rarity, the wide range of signs and • symptoms that can occur, and the • Enlargement of the lymph glands fact that patients may be seen by (also known as lymph nodes) different doctors who are unfamiliar Fluid build-up in the feet and with the syndrome. • ankles The average age of diagnosis is in Increased sweating people in their 50s, however this • can range from 30–80 years old. • Skin changes, including: POEMS syndrome is more common • Thickening of the skin in men than in women. • Red or purple spots on the What are the symptoms surface on the skin of POEMS syndrome? • An increasing amount of hair The most common symptoms on the arms and legs which is result from the peripheral often coarse in texture neuropathy associated with the • Swelling of the fingertips and white nails (known as ‘clubbing’) Infoline: 0800 980 3332 3 • Breathlessness To confirm a diagnosis, your doctor will need to perform a thorough Fatigue • physical examination where they • Headaches or blurred vision will look for: • Reduced sexual function • Skin and hair changes (reduced libido, loss of erections) • Evidence of fluid build-up Patient may also be at increased Enlargement of specific glands risk of deep vein thrombosis (blood • and organs such as lymph clots in the deep veins of the body, glands, liver and/or the spleen typically the legs). Patients may have fewer symptoms at diagnosis • Signs of optic nerve swelling but develop more symptoms over Signs of neuropathy by time as the syndrome progresses. • conducting a complete examination of the nervous How is POEMS syndrome system diagnosed? Your doctor will confirm a diagnosis Not all of the criteria referred through a number of tests and to in the name “POEMS” investigations which include: (polyneuropathy, organomegaly, endocrinopathy, monoclonal 1. Blood tests, which check: plasma cell disorder and skin • Your levels of white blood cells, changes) are always present. red blood cells and platelets POEMS syndrome may be diagnosed if the patient has all of • Your liver and kidney function, the following: and function of your endocrine glands • Polyneuropathy • If paraprotein is present • Monoclonal plasma cell disorder • The level of certain cytokines One or more of the other “major • including VEGF criteria” of the POEMS syndrome (sclerotic bone lesions, raised 2. Your urine may also be tested VEGF and Castleman disease) for the presence of part of the paraprotein called light chains One or more of the other criteria • (also sometimes called Bence (such as hormone changes or Jones Protein) skin changes). 4 myeloma.org.uk 3. X-rays and scans to look for Treatment of POEMS syndrome will abnormal bone growth or bone depend on several factors: damage • Whether the underlying plasma 4. Biopsies and other tests: cell disorder is widespread in the bone marrow, or at a specific • A bone marrow biopsy, or a location (a plasmacytoma) biopsy of any individual bone lesions (plasmacytomas) • The main symptoms present and identified on scans, may be organs affected performed to look for the • Age and general fitness of the presence of abnormal plasma patient cells Treatment may include • A nerve conduction test may chemotherapy and/or radiotherapy. be performed to assess nerve Some patients will be given stem cell function and damage transplantation. The treatments are • A lumbar puncture may be similar to those used in other plasma performed to look for high levels cell conditions, including solitary of protein in the spinal fluid plasmacytoma and myeloma. How is POEMS Radiotherapy syndrome treated? You will typically receive radiotherapy treatment if you have It is important that treatment is an abnormal bone growth in just started as soon as possible, because one or two areas of the bone, but progression of the condition is rapid no evidence of abnormal plasma without treatment. cells spread throughout your bone Treatment improves symptoms marrow. You will be assessed by but may not cure the underlying a radiotherapy specialist who will condition. However, the outlook decide on the exact amount and for patients with treated POEMS number of treatments needed. You syndrome has improved greatly in may be given drug treatment in the last few years. In a study of 100 addition to radiotherapy. POEMS patients in the UK, 10-year survival for patients was 82% (82 in Stem cell transplantation 100 patients). Patients who relapse You may be considered for generally do well after further high-dose therapy and stem cell treatment. transplantation (HDT-SCT) if the Infoline: 0800 980 3332 5 abnormal plasma cells are widely The choice of treatment and dose spread throughout your bone will take into account side effects marrow, and if your age and fitness which can be problematic in POEMS make this suitable for you. You will syndrome (including neuropathy be given high dose chemotherapy and increased risk of thrombosis). (often melphalan) before the stem Treatment is effective in the majority cell transplant, and additional drug of patients, but improvement is treatments before or afterwards. normally gradual. Once there is A reaction called “engraftment evidence of an improvement in syndrome” can occur after HDT-SCT, symptoms, quality of life and day-to- with symptoms such as fever, day functioning, it is recommended weight gain and skin symptoms, that patients are referred for a and is treated with steroids. period of rehabilitation, preferably at a specialist rehabilitation centre, to Drug treatment allow nerve and muscle function to If the abnormal plasma cells are improve. more widely spread throughout your bone marrow, and you are not able to Nerve pain is common in POEMS undergo HDT-SCT, it is likely that you syndrome and is often treated will receive a combination of different with drugs such as amitriptyline,
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