A CASE OF CROW-FUKASE SYNDROME WITH ELEVATED SOLUBLE INTERLEUKIN-6 RECEPTOR IN CEREBROSPINAL-FLUID - RESPONSE TO DOUBLE-FILTRATION PLASMAPHERESIS AND CORTICOSTEROIDS

We report the case of a 33-year-old Japanese male who presented with t hrombocytosis, lower limb edema, severe polyneuropathy with elevated c erebrospinal fluid (CSF) protein level and serum IgA lambda monoclonal component, fulfilling the manifestations of Crow-Fukase syndrome. A h igh level of soluble interleukin-6 receptor in the CSF was also found, which fluctuated in parallel with the clinical course. Initial treatm ent with double-filtration plasmapheresis (DFPP) reduced the serum IgA paraprotein level with improvement of the sensory component of the po lyneuropathy and decrease of soluble interleukin-6 receptor in the CSF . The remaining clinical features waned off after steroid treatment. T he possible role of interleukin-6 in the pathogenesis of the Crow-Fuka se syndrome and the utility of DFPP treatment are discussed.