POEMS Syndrome

Abstract

POEMS syndrome is defined by the presence of a peripheral neuropathy (P), a monoclonal plasma cell disorder (M), and other paraneoplastic features, the most common of which include organomegaly (O), endocrinopathy (E), skin changes (S), papilledema, edema, effusions, ascites, and thrombocytosis. Virtually all patients will have either sclerotic bone lesion(s) or co-existent Castleman's disease. Not all features of the disease are required to make the diagnosis, and early recognition is important to reduce morbidity. Other names for the syndrome include osteosclerotic myeloma, Crow-Fukase syndrome, or Takatsuki syndrome. Because the peripheral neuropathy is frequently the overriding symptom and because the characteristics of the neuropathy are similar to that chronic inflammatory demyelinating polyneuropathy (CIDP), patients are frequently misdiagnosed with CIDP or monoclonal gammopathy of underdetermined significance (MGUS)-associated peripheral neuropathy. Not until additional features of the POEMS syndrome are recognized is the correct diagnosis made and effective therapies initiated. Clues to an early diagnosis include thrombocytosis and sclerotic bone lesions on plain skeletal radiographs. Therapies that may be effective in patients with CIDP and MGUS-associated peripheral neuropathy (intravenous gammaglobulin and plasmapheresis) are not effective in patients with POEMS. Instead, the mainstays of therapy for patients with POEMS include irradiation, corticosteroids, and alkylator-based therapy, including high-dose chemotherapy with peripheral blood stem cell transplantation.

Dangerous small B-cell clones

“…This syndrome has been comprehensively reviewed. 103,104 The minimum features required for diagnosis are peripheral neuropathy, osteosclerotic myeloma, or Castleman disease and at least one of the other features listed earlier.…”

“…PBSCT is emerging as the most effective therapy, resulting in marked and sustained reduction of VEGF coincident with the clinical improvement. 104,108 Scleromyxedema Scleromyxedema is a rare progressive and disabling disease (only 114 cases were reviewed in 2001), 109 characterized by a small IgG monoclonal protein, papular mucinous deposits of 2 to 3 mm in diameter, symmetric, and arranged in a linear array, most commonly located on the face, neck, upper trunk, forearm, and hands. With time the skin stiffens with reduced mobility of the mouth and joints, leading to a scleroderma-like appearance.…”

Restrictive usage of monoclonal immunoglobulin λ light chain germline in POEMS syndrome

“…All patients met the criteria for the diagnosis of POEMS syndrome 17 and had the 5 distinctive features of POEMS. The serum monoclonal immunoglobulin light chain was -type in all patients.…”

Treatment of POEMS syndrome with bevacizumab

“…All symptoms indicated of a relapse. 4 The patient received diuretics, ACE blocker, low dose β blocker and thyroid hormone. Since he met criteria for highly active disease, we started a combined immunochemotherapy with bevacizumab, intravenous cyclophosphamide and oral steroids based on the response reported by Badros et al After two courses, a clinical improvement was seen with resolution of the effusions and improvement of the left ventricular function.…”