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Synonyms: osteosclerotic myeloma, Crow-Fukase syndrome, Takatsuki syndrome
POEMS syndrome is defined as the presence of a peripheral neuropathy, a monoclonal plasma cell disorder, and other paraneoplastic features. The acronym stands for:
- Peripheral neuropathy: the main symptom.
- M protein (or monoclonal gammopathy).
- Skin changes.
Not all these features are required for diagnosis.
Other possible features not represented in the acronym are: sclerotic bone lesions, papilloedema, pleural effusion, oedema, ascites, thrombocytosis and Castleman's disease (giant lymph node hyperplasia).
Both major criteria and at least one minor criterion are required for diagnosis:
- Monoclonal plasma-proliferative disorder.
- Sclerotic bone lesions.
- Castleman's disease.
- Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy).
- Oedema (peripheral oedema, pleural effusion or ascites).
- Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid or pancreatic - but diabetes mellitus or hypothyroidism alone are insufficient, as they are common in the general population).
- Skin changes (hyperpigmentation, hypertrichosis, plethora, haemangiomata, white nails).
- POEMS syndrome is probably rare (of the order of hundreds of cases are reported) but may be under-diagnosed and the incidence is unknown.
- Peak incidence is age 40-60.
The cause is unknown. Increased levels of cytokines, particularly vascular endothelial growth factor (VEGF), may play a role.
Clinical features and complications
These are listed in order of the most common at diagnosis:
- The defining symptom is a chronic peripheral neuropathy, with motor features predominating:
- It starts in the feet with paraesthesia.
- There is symmetrical and proximal spread.
- Progression is usually gradual but can be rapid.
- Respiratory muscles may be affected.
- Some patients also have pain.
- Osteosclerotic lesions:
- Occur in 95% of patients.
- May be solitary or multiple; there may be mixed sclerotic and lytic lesions.
- Skin changes:
- Hyperpigmentation and hypertrichosis are common; coarse black hair may occur on the extremities.
- Other skin changes are: thickening, haemangiomata, plethora, clubbing, white nails.
- Most commonly, hypogonadism.
- Diabetes and hypothyroidism are also common.
- Adrenal insufficiency and hypocalcaemia can occur.
- There may be multiple endocrine axes affected.
- Organomegaly - may be:
- Castleman's disease.
- Affects up to half of patients.
- May be asymptomatic, or may cause headache and visual symptoms.
- Extravascular volume overload - pitting oedema, ascites or pleural effusions.
- Uncommon features or complications are:
- Respiratory - pulmonary hypertension, restrictive lung disease.
- Thrombosis - arterial or venous.
- Renal impairment (rare).
- Congestive cardiac failure.
- A capillary leak-like syndrome.
- Serum proteins investigations:
- By definition, all patients have a monoclonal plasma-proliferative disorder.
- The monoclonal protein is typically small; it may be missed on electrophoresis unless immunofixation is done on both serum and 24-hour urine.
- VEGF levels are nearly always raised in patients with active POEMS syndrome. Other cytokine levels may be raised.
- FBC: there may be thrombocytosis with or without polycythaemia.
- Liver function and renal function.
- A full endocrine assessment is advised if POEMS is strongly suspected.
- Skeletal survey may show sclerotic or lytic bone lesions.
- CXR if there are cardiovascular/respiratory symptoms.
- Nerve conduction studies and electromyography.
- ECG, echocardiogram and lung function tests for cardiorespiratory symptoms.
- Biopsy of bone marrow or an osteosclerotic lesion - may show marrow monoclonal plasma cells.
- Lymph node biopsy - if lymphadenopathy; may demonstrate Castleman's disease.
- Nerve biopsies - usually reveal evidence of axonal degeneration and demyelination.
- Chronic inflammatory demyelinating polyneuropathy.
- Monoclonal gammopathy of undetermined significance with associated peripheral neuropathy.
- Multiple myeloma - may have polyneuropathy and, rarely, can present with diffuse osteosclerotic bone lesions.
- Solitary plasmacytoma of bone - may have a small amount of monoclonal protein in serum or urine.
- Waldenström's macroglobulinaemia.
- The interconnections between POEMS syndrome, osteosclerotic myeloma and Castleman's disease are still under investigation.
- Possible associations include pulmonary hypertension, restrictive lung disease, thrombotic diatheses, arthralgias, cardiomyopathy and low vitamin B12.
- There is currently insufficient evidence regarding the treatment options for POEMS syndrome on which to base practice.
- Supportive care as required for neurological and respiratory problems - eg, physiotherapy, occupational therapy, respiratory support or multidisciplinary care.
- Radiotherapy - if the osteosclerotic lesions are single or restricted to a limited area.
- If osteosclerotic lesions are widespread - chemotherapy or autologous stem cell transplantation.Possible treatments include:
- Melphalan and prednisolone.
- Cyclophosphamide ± prednisolone.
- High-dose chemotherapy with stem cell transplantation.
- Lenalidomide - has been used with some success.
- Thalidomide and bortezomib - may be effective but risk exacerbating peripheral neuropathy. The benefit of anti-VEGF antibodies is conflicting.
The median survival of patients with POEMS syndrome is about 14 years.Prompt recognition and institution of supportive care measures and therapy directed against the plasma cell result in the best outcomes.
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