POEMS Syndrome Follow-up
- Author: Joanna L Chan, MD; Chief Editor: Dirk M Elston, MD more...
Further Outpatient Care
Even if the plasmacytoma completely responds to treatment, patients need long-term follow-up care because some symptoms, such as neurologic defects and functional loss due to tightening of the skin, may be permanent.
The prognosis depends on the extent of the underlying plasma cell disorder and its response to treatment. The prognosis is best for patients with a single lytic lesion. The prognosis is worst for patients with a plasma cell disorder involving the bone marrow. Patients with multiple lytic bone lesions have an intermediate prognosis. When the plasma cell disorder responds to treatment, all other symptoms usually improve or resolve completely. Reduced survival times are associated with the presence of cough, and respiratory weakness decreased mean survival time from 139 months to 87 months in one study.
The morbidity associated with POEMS syndrome depends on the systems involved and ranges from skin pigment alteration to debilitating weakness and loss of function. The median survival period for patients with POEMS syndrome is 8 years. The natural course of POEMS syndrome is chronic, with a reported median survival of approximately a decade (8-13.8 y). In their review, Miralles and colleagues reported a 5-year survival rate of 60%.
Overall shorter survival has been associated with extravascular volume overload (eg, effusions, edema, ascites) and fingernail clubbing. Cardiorespiratory failure, renal failure, infection, and progressive inanition are among the most common causes of death. The neurologic sequelae of POEMS syndrome cause approximately 50% of the patients with POEMS syndrome to become bedridden. Death may also occur as a consequence of decubitus ulcers and thromboses due to inactivity, organomegaly, and endocrinopathy, rather than as a consequence of the aggressiveness of the monoclonal protein.
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