We appreciate the comment of Domingo et al and agree that the article by Nakanishi and coworkers1 reviewing the Crow-Fukase syndrome in Japan provides a good review and can be compared with the cases of POEMS reported outside Japan. It is interesting to note that many of the patients in Japan with Crow-Fukase syndrome do not have multiple myeloma, a presentation somewhat different from the typical patients with POEMS syndrome. We were unable to determine from the review by Nakanishi et al whether the three patients reported in their series with IgA κ-light-chain disease had the constellation of findings that would have classified them as a complete POEMS syndrome, as our patient. It is, nevertheless, interesting that the incidence of κ-chain polyneuropathy occurs so rarely. Coagulopathy occurs in approximately 15% of patients with multiple myeloma; the incidence and characteristics of the coagulopathy associated with POEMS need further study.
Westrick MA, Hyman BT. Multiple Myeloma With Polyneuropathy and Coagulopathy-Reply. Arch Intern Med. 1986;146(10):2089. doi:10.1001/archinte.1986.00360220271054