POEMS syndrome

POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin

changes) is characterized by the presence of a monoclonal plasma cell disorder, peripheral
neuropathy, and one or more of the following features: osteosclerotic myeloma, Castleman's
disease, increased levels of serum vascular endothelial growth factor (VEGF), organomegaly,
endocrinopathy, edema, typical skin changes, and papilledema.

CLINICAL FEATURES — The clinical manifestations of POEMS syndrome are protean. By

definition, all patients have peripheral neuropathy and a monoclonal plasma cell disorder,
almost always of the lambda light chain type. In addition, almost all patients have either
osteosclerotic myeloma (solitary or multiple) or Castleman's disease. One should therefore be
cautious about diagnosing POEMS syndrome in the absence of either osteosclerotic lesions or
Castleman's disease. The prevalence of other manifestations (eg, organomegaly,
endocrinopathy, skin changes, edema, and papilledema) varies greatly 

Peripheral neuropathy and CNS involvement — 

Peripheral neuropathy is required for the diagnosis of POEMS syndrome, and usually dominates
the clinical picture.
Symptoms begin in the feet and consist of tingling, paresthesias, and feelings of coldness.
Motor involvement follows the sensory symptoms.
Both are distal, symmetric, and progressive, with a gradual proximal spread.
Severe weakness occurs in more than half of patients and results in an inability to climb stairs,
arise from a chair, or grip objects firmly with the hands, consistent with a predominantly motor
chronic inflammatory demyelinating polyneuropathy.
The course is progressive and patients may be confined to a wheelchair.
Autonomic symptoms are not a feature.
Physical examination reveals a symmetric sensorimotor neuropathy involving the extremities.
Muscle weakness is more marked than sensory loss.
Touch, pressure, vibratory, and joint position senses are often involved.
Less frequently, loss of temperature discrimination and nociception occurs.
Cranial nerves are not affected.
Cerebrospinal fluid protein levels are increased in virtually all patients, with more than half of
patients having a cerebrospinal fluid protein level >100 mg/dL.

Electromyographic studies show slowing of nerve conduction, prolonged distal latencies, and
severe attenuation of compound muscle action potentials. Conduction block is rarely found, but
slowing of motor conduction is proportionately greater than the reduction in the compound
muscle action potential amplitude. Distal fibrillation potentials are found on needle
electromyography.
Criteria for the diagnosis of POEMS syndrome

Mandatory major criteria (both required)

Polyneuropathy

Monoclonal plasma cell proliferative disorder (almost always lambda)

Other major criteria (one required)

Sclerotic bone lesions

Castleman's disease

Elevated levels of vascular endothelial growth factor (VEGF)*

Minor criteria (one required)

Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)

Extravascular volume overload (edema, pleural effusion, or ascites)

Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)•

Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora,

acrocyanosis, flushing, white nails)

Papilledema

Thrombocytosis/polycythemia