The POEMS syndrome among Chinese: association with Castleman's disease and some immunological abnormalities

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.