POEMS Syndrome

What is POEMS Syndrome?

POEMS syndrome is a rare paraneoplastic syndrome, meaning it is caused by an underlying plasma cell neoplasm (an abnormal growth of antibody-making cells).^{[1, 2]} The name stands for polyneuropathy, organomegaly (enlarged organs), endocrinopathy (hormone problems), monoclonal protein and skin changes, though many other features can occur.^{[1, 3]} It needs diagnosis and treatment by physicians.

Common symptoms

Nerve damage is the central feature. It is a demyelinating neuropathy that can start over weeks or months, can be painful, and can become disabling quickly.^[2] Other features include sclerotic (hardened) bone lesions, enlarged organs, hormone problems, characteristic skin changes, swelling from fluid, and a high platelet count.^[1]

Further manifestations include fluid in the abdomen, blood clots in veins or arteries, and problems of the kidneys, lungs and heart.^[3] Optic nerve swelling (papilledema) can also occur.^[1]

Causes and risk factors

The cause is not well understood.^[1] Chronic overproduction of inflammatory signaling molecules appears to contribute.^[4] Vascular endothelial growth factor (VEGF) is raised in the disease and is useful for diagnosis and monitoring, although treatment aimed at VEGF alone has been disappointing, which suggests that other inflammatory processes also matter.^[2]

How it is diagnosed and treated conventionally

Diagnosis is often delayed because the syndrome is rare and can be mistaken for other nerve disorders, most often chronic inflammatory demyelinating polyradiculoneuropathy.^{[1, 3]} It requires both a polyradiculoneuropathy and a clonal plasma cell disorder, plus other major and minor criteria.^[1]

Treatment depends on how widespread the disease is. A single dominant bone lesion is treated first with radiation, while diffuse lesions or bone marrow involvement call for systemic therapy.^[1] Alkylator drugs are the mainstay, given as low-dose therapy or high-dose therapy with stem cell transplant, and lenalidomide and bortezomib look promising.^{[1, 3]} Thalidomide and bortezomib have to be weighed against their risk of making the neuropathy worse.^[1] Early treatment, before severe nerve damage develops, together with supportive care such as physiotherapy, is important for neurological recovery.^[3]

Why POEMS Syndrome calls for a whole-person approach

POEMS syndrome affects nerves, hormones, skin, fluid balance and blood at once, so no single symptom tells the whole story. Treatment of the plasma cell disorder is the priority, and supportive measures are best added once that treatment is in place.^[1]

Supportive care here is limited to the symptoms and strains around the disease, always with your treating team informed.

Nerve pain and weakness

The neuropathy can be painful and rapidly disabling.^[2] Physiotherapy is part of recommended supportive care.^[3] In a review of exercise for neuropathic pain from other causes, exercise reduced pain intensity, and experts recommended it as a complementary therapy.^[5]

Treatment effects

Some drugs used against the disease can themselves harm nerves, so symptoms may reflect either the disease or its treatment.^[1] Reporting new or changing symptoms to your physician matters.

Hormones and fatigue

Endocrine problems are one of the defining features of the disease.^[1] Fatigue and weakness are common and can have several causes, which your physicians need to sort out.

Stress and coping with a rare disease

Living with a rare and disabling condition, and a delayed diagnosis, can bring stress and worry. Stress management is a reasonable goal for supportive care.

Acupuncture as supportive care in POEMS Syndrome

What the research shows

A PubMed search found no clinical trials or systematic reviews of acupuncture for POEMS syndrome. We cannot say that acupuncture helps the disease or its neuropathy.

The nearest research is in multiple myeloma, another plasma cell disorder. A systematic review of five studies (97 patients) found pain scores fell after acupuncture for drug-related neuropathy, with no severe adverse effects, but the studies varied widely and no study had looked at neuropathy caused by the disease itself.^[6]

A network meta-analysis of 33 studies (2,027 participants) in chemotherapy-induced nerve damage found acupuncture-related treatments improved symptoms, pain and quality of life compared with usual care or medicines.^[7] A review of randomized trials of acupuncture in neuropathy of other causes, including diabetes, found benefit in some, and said more rigorous studies with sham controls were needed.^[8] An overview of 51 systematic reviews reported benefit for cancer-related pain, fatigue and insomnia, although most of the reviews were of very low quality.^[9]

These results come from other conditions, so they may not carry over to POEMS syndrome.

How acupuncture may work

No studies explain how acupuncture might act in POEMS syndrome. We do not claim that it affects the plasma cells, VEGF or the underlying disease.

What treatment involves

Treatment uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes. A course of several visits is followed by reassessment. The aim would be comfort, sleep and coping with symptoms.

In prospective studies, about 9 in 100 patients had a minor reaction such as bleeding, soreness or redness at a needle site, and serious events occurred in about 1 in 10,000 patients.^[10] Tell the practitioner first if you have a bleeding disorder, take blood thinners, are pregnant, or have a pacemaker. Also tell us about blood clots, swelling, skin changes or numb areas, since POEMS syndrome can involve all of these and needle sites need extra care.

Ayurvedic medicine as supportive care in POEMS Syndrome

The Ayurvedic view

Ayurveda has no classical name that matches POEMS syndrome. Practitioners may describe nerve symptoms in terms of Vata imbalance and swelling or skin changes in terms of Kapha or Pitta. This is a traditional frame, not a medical diagnosis.

Therapies that may be used

Care is individualized and traditionally includes:

• Diet and daily routine, such as regular meals and steady sleep hours, adapted to any restrictions from your physicians
• Gentle relaxation and breathing practices for stress and sleep
• Gentle oil massage for comfort, avoiding numb, swollen or fragile skin
• Herbs, only if your physicians agree and only prescribed individually by a qualified practitioner

Panchakarma is a traditional set of cleansing procedures. It is not appropriate during pregnancy, acute illness or frailty, and it is not suitable during chemotherapy or stem cell transplant, or with nerve, kidney, heart or clotting problems.

What the research shows

No clinical trials of Ayurvedic treatment for POEMS syndrome were found, so the evidence for the disease is none.

Some evidence exists for stress in other groups. A meta-analysis of 12 trials found ashwagandha reduced anxiety and stress compared with placebo, though the certainty was low.^[11] Those trials did not include people with POEMS syndrome or on chemotherapy, so it should only be considered with your hematologist's knowledge. A review of integrative care in multiple myeloma notes that many patients use supplements on their own, so it is important to know what they do.^[12]

Herbal medicine as supportive care in POEMS Syndrome

Herbs and formulas that have been studied

No herbs or herbal formulas have been studied in clinical trials for POEMS syndrome. We do not name any herb as a treatment for it.

What the research shows

The evidence for herbal medicine in this disease is none. Because the disease is serious and treated with powerful drugs, we do not use herbs to treat it. Any herb considered is for a symptom such as poor sleep, and only after your treating team has reviewed it.

Safety and herb-drug interactions

Herbs can interact with prescribed drugs, and a review of herb-drug interactions found that serious consequences can occur.^[13] People with POEMS syndrome may take anticoagulants because of clot risk, and case reports describe bleeding when warfarin is combined with herbs including dan shen, dang gui, ginger, ginseng and Boswellia.^[14] Tell us about every drug you take, including chemotherapy, thalidomide-related drugs and steroids.

Herbal and dietary supplements account for about 20% of cases of drug-related liver injury in the United States.^[15] About one fifth of Ayurvedic products bought online contained detectable lead, mercury or arsenic, so products must come from tested sources.^[16] Tell us if you are pregnant or breastfeeding, or have liver or kidney disease.

Translating traditional medicine into modern biological language

This section is interpretation, not equivalence. Chinese medicine may describe numbness, weakness and swelling as obstruction of qi and blood with dampness. Ayurveda may describe nerve symptoms as Vata disturbance. Researchers studying symptoms of this kind look at things that loosely parallel these ideas, such as nerve pain, sleep and the stress response, but no study has linked them to POEMS syndrome.

The biological process in POEMS syndrome is a clonal plasma cell disorder with raised VEGF and inflammatory signaling.^{[1, 4]} No study shows that a TCM pattern or a dosha corresponds to that process.

How this care fits with your medical care

Supportive care here sits alongside your treating team. POEMS syndrome needs specialist-directed treatment of the plasma cell disorder, and early treatment gives the best neurological outcomes.^[3] Never stop, delay or change radiation, chemotherapy, steroids, anticoagulants or any other prescribed treatment on your own. We do not diagnose or manage the disease, change prescriptions, or replace your physicians.

For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Please bring your diagnosis, a full list of medicines and supplements, your treatment plan, and recent blood test results.

When to seek urgent medical care

POEMS syndrome can progress quickly and can involve clots, fluid collections and organ impairment.^[3]

• Rapidly worsening weakness, trouble walking or trouble swallowing
• Shortness of breath, chest pain or fainting
• A swollen, painful or red limb, which can signal a blood clot
• Sudden vision changes or severe headache
• Severe abdominal swelling or pain, or sharply reduced urine
• Fever with signs of infection during chemotherapy or while taking steroids

If you have any of these, call 911 or go to the nearest emergency department.