POEMS syndrome, a rare paraneoplastic condition, occurs when the body erroneously targets normal nervous system cells in response to a malignant tumor. The disorder's five main symptoms are polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities. Symptoms include weakening arm and leg nerves (sensorimotor polyneuropathy), hepatosplenomegaly, lymphadenitis, hyperpigmentation, thicker skin, and fast hair growth. Men are 1.5 times more likely than women to develop POEMS syndrome, which usually starts in their 50s. POEMS syndrome's etiology is unknown, however research suggests that VEGF (vascular endothelial growth factor) is involved.

POEMS syndrome is chronic, and the prognosis varies on disease severity and therapy response. There is no conventional POEMS syndrome treatment. Hematopoietic cell transplantation, chemotherapy, and radiation are treatments. POEMS syndrome patients live over 14 years on average.

Signs & Symptoms

Symptoms POEMS syndrome affects multiple systems. Polyneuropathy is frequent and causes weakness, numbness, tingling, paresthesia, and impaired muscular responsiveness in the toes, feet, legs, and hands. Hepatomegaly, splenomegaly, and lymphadenopathy are organomegaly symptoms. Castleman disease and angiofollicular lymph node hyperplasia enlarge lymph nodes.

Skin problems include hyperpigmentation, hypertrichosis, scleroderma, hyperhidrosis, acrocyanosis, leukonychia, cherry angiomata, clubbed fingernails, and temple fat loss Polycythemia, thrombocytosis, artery and vein clots, and pulmonary arterial hypertension are other blood disorders.

POEMS syndrome involves pleural effusion, respiratory failure, and ventilatory dysfunction. An expanded optic nerve in the rear of the eye can cause vision problems, headaches, nausea, papilledema, pericardial effusion, edema, ascites, weight loss, and weariness.

Causes

The exact cause of POEMS syndrome is unknown. It is linked to an overproduction of molecules that promote inflammation via cell-to-cell communication (pro-inflammatory cytokines). Researchers have found that people with POEMS syndrome have blood levels that are abnormally high in vascular endothelial growth factor (VEGF) and pro-inflammatory cytokines, such as interleukin-6, interleukin-1, and TNF-alpha. The exact role of cytokines and VEGF in the development of POEMS syndrome requires further research.

Affected Populations

POEMS syndrome affects 1.5 times more men than women, with disease onset usually occurring in patients in their 40s to 60s. POEMS syndrome often goes unrecognized, making it difficult to determine the exact number of people afflicted with the disease.

Diagnosis

Patients suspected of having POEMS syndrome should undergo a thorough clinical evaluation, including a physical exam, a medical history review, and laboratory testing. Confirmation of certain immunologic abnormalities is essential to establishing the diagnosis. Laboratory tests conducted on blood (serum) or cerebrospinal fluid (CSF) may reveal elevated levels of M-proteins, and blood plasma may show high levels of VEGF.

Treatment

POEMS syndrome patients may experience a temporary or permanent remission of symptoms after ionizing radiation (radiotherapy) or surgical removal (excision) of localized osteosclerotic lesions. Patients with widespread disease may benefit from alkylating chemotherapy agents.