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Prepublished online as a Blood First Edition Paper on November 27, 2002; DOI 10.1182/blood-2002-07-2299.
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Blood, 1 April 2003, Vol. 101, No. 7, pp. 2496-2506
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
POEMS syndrome: definitions and long-term
outcome
Angela Dispenzieri,
Robert A. Kyle,
Martha Q. Lacy,
S. Vincent Rajkumar,
Terry M. Therneau,
Dirk R. Larson,
Philip R. Greipp,
Thomas E. Witzig,
Rita Basu,
Guillermo A. Suarez,
Rafael Fonseca,
John A. Lust, and
Morie A. Gertz
From the Division of Hematology and Internal Medicine;
Section of Biostatistics; Division of Endocrinology, Diabetes,
Metabolism, Nutrition, and Internal Medicine; and Department of
Neurology; Mayo Clinic, Rochester, MN.
The POEMS syndrome (coined to refer to polyneuropathy,
organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to
establish the diagnosis, treatment efficacy, and prognosis. We
identified 99 patients with POEMS syndrome. Minimal criteria were a
sensorimotor peripheral neuropathy and evidence of a monoclonal plasmaproliferative disorder. To distinguish POEMS from neuropathy associated with monoclonal gammopathy of undetermined significance, additional criteria were included: a bone lesion, Castleman disease, organomegaly (or lymphadenopathy), endocrinopathy, edema (peripheral edema, ascites, or effusions), and skin changes. The median age at
presentation was 51 years; 63% were men. Median survival was 165 months. With the exception of fingernail clubbing
(P = .03) and extravascular volume overload
(P = .04), no presenting feature, including the number of
presenting features, was predictive of survival. Response to therapy
(P < .001) was predictive of survival. Pulmonary
hypertension, renal failure, thrombotic events, and congestive heart
failure were observed and appear to be part of the syndrome. In 18 patients (18%), new disease manifestations developed over time. More
than 50% of patients had a response to radiation, and 22% to 50% had
responses to prednisone and a combination of melphalan and prednisone,
respectively. We conclude that the median survival of patients with
POEMS syndrome is 165 months, independent of the number of syndrome
features, bone lesions, or plasma cells at diagnosis. Additional
features of the syndrome often develop, but the complications of
classic multiple myeloma rarely develop.
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