Serum immunoglobulin free light chain measurements and heavy chain isotype usage provide insight into disease biology in patients with POEMS syndrome

Trista Stankowski-Drengler, Morie A. Gertz, Jerry A. Katzmann, Martha Q. Lacy, Shaji Kumar, Nelson Leung, Suzanne R. Hayman, Francis Buadi, Robert A. Kyle, S. Vincent Rajkumar, Angela Dispenzieri

Research output: Contribution to journalArticle

37 Scopus citations

Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) syndrome is a rare paraneoplastic syndrome in which nearly all patients have a monoclonal lambda restricted plasma cell disorder. We investigated whether patients with POEMS have abnormal serum immunoglobulin free light chain (FLC) ratios. Fifty patients with newly diagnosed POEMS syndrome were assessed. Cystatin C levels were measured to discern whether subclinical renal insufficiency could account for FLC elevations in the presence of a normal FLC ratio. Forty-five patients (90%) had elevated lambda FLC; however, only nine (18%) had abnormal FLC ratios. The rise in serum FLC of POEMS patients appeared to be multifactorial - both a function of subclinical renal insufficiency and polyclonal activation of medullary and extramedullary plasma cells. Those patients expressing a clonal IgA were more likely to have clonal plasmacytosis observed on iliac crest biopsy than those with IgG. In summary, serum immunoglobulin profiles are unique in POEMS syndrome as compared with other plasma cell disorders.

Original languageEnglish (US)
Pages (from-to)431-434
Number of pages4
JournalAmerican journal of hematology
Volume85
Issue number6
DOIs
StatePublished - Jun 2010

ASJC Scopus subject areas

  • Hematology

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