Clinicopathologic analysis of POEMS syndrome and related diseases

Clin Lymphoma Myeloma Leuk. 2015 Jan;15(1):e15-21. doi: 10.1016/j.clml.2014.04.017. Epub 2014 Jul 23.

Abstract

Background: POEMS syndrome, a rare paraneoplastic disease, is related to multiple organs, multiple systems, and multiple disciplines and can be mistaken for other disorders. Consequently, the diagnoses are often delayed. In this work we studied the clinicopathologic characteristics of the POEMS syndrome to improve early diagnosis to prevent irreversible damage.

Patients and methods: We conducted a clinicopathologic analysis of 9 cases of POEMS and made a differential diagnosis with related diseases.

Results: The patients with POEMS syndrome were shown to have complicated clinical characteristics, including peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma proliferative disorder, skin changes, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease. POEMS syndrome shared many elements with other diseases and the key way to differentiate them was to determine whether there were other fundamental POEMS syndrome symptoms or signs. The level of M-protein in serum and plasma cells in bone marrow of POEMS patients was lower than that of patients with multiple myeloma (MM). Sclerotic bone lesions were a distinctive feature in patients with POEMS, compared with in those with MM.

Conclusion: Some unique clinicopathologic characteristics of POEMS syndrome can be used for differential diagnosis. This study provides increased awareness of POEMS syndrome.

Keywords: Clinicopathologic characteristics; Differential diagnosis; Multiple myeloma; Osteosclerotic myeloma; Sclerotic bone lesions.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Bone Marrow / pathology
  • Bone and Bones / pathology
  • Diagnosis, Differential
  • Female
  • Humans
  • Lymph Nodes / pathology
  • Male
  • Middle Aged
  • Multiple Myeloma / diagnosis
  • POEMS Syndrome / diagnosis*
  • POEMS Syndrome / pathology
  • Peripheral Nervous System Diseases / diagnosis
  • Pleural Effusion / diagnostic imaging
  • Skin / pathology
  • Tomography, X-Ray Computed