POEMS syndrome

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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POEMS syndrome
Synonyms	Crow–Fukase syndrome, Takatsuki disease
Pronounce
Specialty	Hematology, Neurology
Symptoms	Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes
Complications	N/A
Onset	Middle age
Duration	Chronic
Types	N/A
Causes	Unknown, associated with monoclonal plasma cell disorder
Risks
Diagnosis	Clinical evaluation, blood tests, imaging studies, biopsy
Differential diagnosis	Multiple myeloma, Chronic inflammatory demyelinating polyneuropathy, Amyloidosis
Prevention	N/A
Treatment	Radiation therapy, Chemotherapy, Corticosteroids, Stem cell transplantation
Medication	N/A
Prognosis	Variable, depends on response to treatment
Frequency	Rare
Deaths	N/A

POEMS syndrome is a rare paraneoplastic syndrome that is associated with an underlying plasma cell disorder. The acronym "POEMS" stands for the five main features of the syndrome: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes.

Clinical Features

Polyneuropathy

Polyneuropathy in POEMS syndrome is typically a symmetric, demyelinating neuropathy that affects both sensory and motor nerves. Patients often present with weakness, numbness, and tingling in the extremities.

Organomegaly

Organomegaly refers to the abnormal enlargement of organs. In POEMS syndrome, this often includes hepatomegaly (enlarged liver), splenomegaly (enlarged spleen), and lymphadenopathy (enlarged lymph nodes).

Endocrinopathy

Endocrinopathy in POEMS syndrome can involve multiple endocrine glands. Common manifestations include hypogonadism, hypothyroidism, and diabetes mellitus.

Monoclonal Plasma Cell Disorder

The presence of a monoclonal plasma cell disorder is a hallmark of POEMS syndrome. This is often detected as a monoclonal protein in the blood or urine, and may be associated with osteosclerotic myeloma.

Skin Changes

Skin changes in POEMS syndrome can include hyperpigmentation, hemangiomas, and hypertrichosis. These changes are often noticeable and can be a key diagnostic feature.

Pathophysiology

The exact pathophysiology of POEMS syndrome is not fully understood, but it is believed to be related to the overproduction of cytokines by the abnormal plasma cells. Vascular endothelial growth factor (VEGF) is one cytokine that is often elevated in patients with POEMS syndrome and may play a role in the disease manifestations.

Diagnosis

The diagnosis of POEMS syndrome is based on the presence of the major criteria (polyneuropathy and monoclonal plasma cell disorder) along with at least one of the minor criteria (organomegaly, endocrinopathy, skin changes, etc.). Additional tests may include electromyography (EMG), nerve conduction studies, and bone marrow biopsy.

Treatment

Treatment of POEMS syndrome often involves addressing the underlying plasma cell disorder. This can include therapies such as radiation therapy, chemotherapy, and autologous stem cell transplantation. Supportive care for symptoms such as neuropathy and endocrinopathy is also important.

Prognosis

The prognosis for patients with POEMS syndrome varies depending on the severity of the disease and the response to treatment. Early diagnosis and treatment can improve outcomes.

See Also

• Paraneoplastic syndrome
• Multiple myeloma
• Peripheral neuropathy