A plasma cell dyscrasia (bone marrow disorder) that
causes multisystem disorders, including neuropathy, organ overgrowth,
endocrine dysfunctions, and skin changes. PEP is an acronym that stands for
Plasma Cell Dyscrasia, Endocrinopathy,
Polyneuropathy Organomegaly Endocrine Monoclonal
Protein Skin Lesions Syndrome; POEMS Syndrome; Osteosclerotic Myeloma.
Mainly affects adults in the 45to 65-year-old age
No genetic inheritance demonstrated. Probably
an inflammatory disease.
Pathophysiology is not fully elucidated. Chronic
inflammatory process with high circulating cytokines (interleukin [IL]-1,
IL-6, tumor necrosis factor) and coagulation activator levels.
Parainflammatory axonal demyelinization with polyneuropathy.
Association of polyneuropathy, organomegaly,
endocrinopathy, monoclonal protein plasma cell dyscrasia, and skin changes.
Diagnosis based on monoclonal plasma cell demonstration in bone marrow
The clinical picture is dominated by a
demyelinating polyneuropathy with motor disability and sclerotic skeletal
lesions. Cranial nerves and autonomic nervous system usually unaffected.
Hepatomegaly and sometimes splenomegaly and lymph node enlargement.
Endocrine involvement mainly affects gonadotrophins and androgens.
Hemostasis studies shows procoagulant activity and thrombocytosis. Rarely
associated with fractures and renal involvement. If localized sclerotic
lesions are found, radiotherapy may improve systemic polyneuropathy and if
widespread chemotherapy (melphalan and steroids) is helpful. Survival at 5
years is 60%.
Mainly dominated by a potentially
severe pulmonary hypertension and recurrent cerebrovascular insults. Check
coagulation tests before and define anticoagulation strategy. Check plasma
calcium. Obtain preoperative neurologic evaluation.
Because of vascular abnormalities and
potential organ ischemia, avoid hypotension. Specific considerations in
presence of severe pulmonary hypertension must apply.
Interaction between anesthetic
medications and cerebroinvasive and antihypertensive medications will be
Lesprit P, Godeau B, Authier FJ, et al: Pulmonary hypertension in POEMS
syndrome: A new feature mediated by cytokines. Am J Respir Crit Care Med