The POEMS syndrome among Chinese: association with Castleman''s disease and some immunological abnormalities |
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Authors: | P. Thajeb C. Y. Chee S. F. Lo N. Lee |
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Affiliation: | Section of Neurology, Cathay General Hospital, Taipei, Taiwan. |
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Abstract: | POEMS or Crow-Fukase syndrome is a multisystemic, clinically malignant disorder of obscure etiology. Peripheral neuropathy and plasma cell dyscrasia are central features. The authors now report 7 Chinese patients with this syndrome in which PCD or paraproteinemia were absent in 6, and 2 had a lymph node histology resembling that of hyaline-vascular Castleman's disease. Immunological abnormalities consisted of either increased or decreased numbers of B- and T-cells in 2 cases, and an elevated OKT4/OKT8 ratio with paradoxical dissociation of the lymphocyte transformations to various concentrations and types of mitogens in 1 case. This suggests that the underlying abnormalities of POEMS syndrome are heterogeneous and that it may be an immunologically related syndrome of varying etiology. |
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Keywords: | POEMS syndrome Castleman's disease immunological abnormalities Crow-Fukase syndrome |
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