POEMS syndrome: importance of the clinical laboratory practitioner's role.

BACKGROUND

Polyneuropathy organomegaly, endocrinopathy, monoclonal (M) gammopathy, skin syndrome (POEMS) may be difficult to diagnose as a result of methodological and clinical idiosyncrasies. Four of eleven criteria (M-protein, VEGF, endocrinopathy and thrombocytosis/polycythemia) are closely associated with clinical laboratory testing. POEMS has been largely associated with λ-M-gammopathies. Vascular endothelial growth factor (VEGF) is a recent addition to the major diagnostic criteria. VEGF may alter vascular permeability causing some manifestations of POEMS.

METHODS

Review of the literature that focuses on clinical laboratory issues--endocrinological findings, identification of monoclonal gammopathies by electrophoresis and case demonstration.

RESULTS

Based on the criterion of VEGF, POEMS was diagnosed in a patient with a κ-M-gammopathy.

CONCLUSIONS

Low-level IgA monoclonal proteins that are common in POEMS are often difficult to identify by serum electrophoresis (SPE). Immunofixation electrophoresis is required when polyneuropaties are investigated and an M-protein is not identified by SPE. VEGF may improve the sensitivity for diagnosis of POEMS and findings from capillary leak syndrome which are also associated with elevated VEGF and M-gammopathy suggests that κ-M-gammopathies may be implicated more often. It is demonstrated that due to computerized records, the laboratory practitioner is well suited to help the clinician make this complicated diagnosis.