The DOI matches its Crossref record; 19 of the 19 references it cites were found in a registry or an index.
POEMS syndrome: definitions and long-term outcome
Open accessAbstract
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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Cited by
International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma
Rajkumar, S. V., Dimopoulos, M. A., Palumbo, A., Blade, J., Merlini, G., Mateos, M.-V., Kumar, S., Hillengass, J., Kastritis, E., Richardson, P., et al. (2014) · The Lancet Oncology · doi:10.1016/s1470-2045(14)70442-5 · Cited by 4,978
Self-citationInternational uniform response criteria for multiple myeloma
Durie, B. G. M., Harousseau, J.-L., Miguel, J. S., Bladé, J., Barlogie, B., Anderson, K., Gertz, M., Dimopoulos, M., Westin, J., Sonneveld, P., et al. (2006) · Leukemia · doi:10.1038/sj.leu.2404284 · Cited by 2,632
Self-citationCriteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma
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Self-citationDispenzieri, A., & Fajgenbaum, D. C. (2020) · Blood · doi:10.1182/blood.2019000931 · Cited by 487
Self-citationMultiple myeloma: 2011 update on diagnosis, risk-stratification, and management
Rajkumar, S. V. (2011) · American Journal of Hematology · doi:10.1002/ajh.21913 · Cited by 152
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Metadata
- Authors
- Dispenzieri, A., Kyle, R. A., Lacy, M. Q., Rajkumar, S. V., Therneau, T. M., Larson, D. R., Greipp, P. R., Witzig, T. E., Basu, R., Suarez, G. A., Fonseca, R., Lust, J. A., & Gertz, M. A.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2003-04-01
- Volume
- 101
- Issue
- 7
- Pages
- 2496-2506
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W2166161087
- Cited by
- 820
- References
- 19
- FWCI
- 14.35
- Type
- journal article
- Language
- en
- Open access
- bronze
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