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

Acta Neurol Scand. 1989 Dec;80(6):492-500. doi: 10.1111/j.1600-0404.1989.tb03916.x.

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.

MeSH terms

  • Adult
  • China
  • Endocrine System Diseases / complications
  • Endocrine System Diseases / epidemiology
  • Endocrine System Diseases / ethnology*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Paraproteinemias / complications
  • Paraproteinemias / epidemiology
  • Paraproteinemias / ethnology*
  • Peripheral Nervous System Diseases / complications
  • Peripheral Nervous System Diseases / epidemiology
  • Peripheral Nervous System Diseases / ethnology*
  • Skin Diseases / complications
  • Skin Diseases / epidemiology
  • Skin Diseases / ethnology*
  • Syndrome