POEMS syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]Sabawoon Mirwais, M.B.B.S, M.D.[3]

Overview

POEMS syndrome presents as a chronic progressive polyneuropathy. Patients may have coexisting multi-organ system disorders. The neuropathy is usually symmetrical and ascending. Endocrinopathy, present in majority of cases, involves hypogonadism, hypothyroidism, and abnormalities of the pituitary-adrenal axis. If left untreated, patients suffering from POEMS syndrome may become wheel chair bound. Complications of POEMS syndrome include renal failure, pulmonary hypertension, pathologic fractures, ischemic stroke, restrictive lung disease, polycythemia, thrombocytosis, papilledema, and myocardial infarction. 3, 5, and 10 year overall survival (OS) for patients newly diagnosed with POEMS syndrome is 87.7%, 84.1%, and 77%. Age >50 years, pulmonary hypertension, pleural effusion, and estimated glomerular filtration rate <30 ml/min/1.73 m² were associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model based on randomized sample splitting of 362 patients. Low albumin (defined as<3.2 g/dl) at diagnosis and failure to achieve a complete hematologic response to first-line therapy can be independent risk factors for progression-free survival (PFS). Improvement of plasma cell dyscrasia leads to improvement or marked reduction in other abnormalities. Neuropathy, stroke, and myocardial infarction are the most common causes of death in POEMS syndrome.

Natural History

• POEMS syndrome typically presents as a chronic progressive polyneuropathy with both sensory and motor disability, the motor component is usually present to a greater degree than the sensory component of the disease.
• Many patients may have coexisting multi-organ system disorders such as organomegaly (liver, spleen and lymph nodes), edema, endocrinopathy (pituitary, thyroid, adrenal glands), thrombocytosis, polycythemia, pulmonary hypertension, an M-spike (M protein or para-protein, may be IgA lambda component, IgG lambda component or light chain lambda component) and skin changes. The skin changes include angiomata, hyperpigmentation, hypertrichosis.
• The neuropathy is usually symmetrical and ascending, with either insidious or rapidly progressing onset. Sensory neuropathy precedes motor dysfunction and pin-prick and vibratory sensations are typically affected.
• The endocrinopathy is present in a majority of cases of POEMS syndrome. Almost all patients exhibiting endocrinopathy have hyperestrogenemia. The increased levels of estrogen may be the cause of hypogonadism and impotence seen in male patients. Hypogonadism, hypothyroidism, and abnormalities of the pituitary-adrenal axis are noted in order of descending frequency.
• POEMS syndrome may also lead to glomerular disease (membranoproliferative glomerulonephritis) eventually resulting in renal failure.
• If left untreated, patients suffering from POEMS syndrome may become wheel chair bound.

Complications

• Patients suffering from POEMS syndrome may develop the following complications:
  • Renal failure
  • Pulmonary hypertension
  • Pathological fractures (due to lytic/sclerotic bone lesions)
  • Ischemic stroke
  • Restrictive lung disease
  • Thrombocytosis
  • Arterial and venous thromboses
  • Polycythemia
  • Papilledema
  • Myocardial infarction

Prognosis

• The median survival of patients with POEMS syndrome is 165 months.
• 3, 5, and 10 year overall survival (OS) for patients newly diagnosed with POEMS syndrome is 87.7%, 84.1%, and 77%.
• Age >50 years, pulmonary hypertension, pleural effusion, and estimated glomerular filtration rate <30 ml/min/1.73 m² were associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model based on randomized sample splitting of 362 patients.
• Low albumin (defined as<3.2 g/dl) at diagnosis and failure to achieve a complete hematologic response to first-line therapy can be independent risk factors for progression-free survival (PFS).
• Papilloedema, decreased diffusing capacity of the lung for carbon monoxide (DLCO) and treatment with corticosteroids alone are also related to poor prognosis.
• Improvement of plasma cell dyscrasia leads to improvement or marked reduction in other abnormalities.
• Neuropathy, stroke and myocardial infarction are the most common causes of death in POEMS syndrome.