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Serum immunoglobulin free light chain measurements and heavy chain isotype usage provide insight into disease biology in patients with POEMS syndrome
Author(s) -
StankowskiDrengler Trista,
Gertz Morie A.,
Katzmann Jerry A,
Lacy Martha Q.,
Kumar Shaji,
Leung Nelson,
Hayman Suzanne R.,
Buadi Francis,
Kyle Robert A.,
Rajkumar S. Vincent,
Dispenzieri Angela
Publication year - 2010
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21707
Subject(s) - poems syndrome , organomegaly , plasma cell , polyneuropathy , plasmacytosis , subclinical infection , medicine , immunoglobulin light chain , pathology , immunology , gastroenterology , castleman disease , antibody , monoclonal , disease , multiple myeloma , monoclonal antibody
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. Am. J. Hematol. 2010. © 2010 Wiley‐Liss, Inc.