Ｓｊｏｅｇｒｅｎ症候群を合併した家族性Ｐｅｌｇｅｒ‐Ｈｕｅｔ核異常の１例

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Although familial Pelger-Hüet anomaly has been known as usually asymptomatic, a case of systemic lupus erythematosus associating with this anomaly appears in the literature.This paper reports on a 32-year-old female with famillal Pelger-Hüet anomaly who had clinical symptoms of Sjögren's syndrome and was positive for antibodies to SS-A. Immunological studies of the patient's peripheral lymphocytes disclosed decreased number of OKT8 cells and diminished blastoid transformation induced both by PHA and ConA.Judging from the fact that other authors also observed immunological abnormalities accompanying Pelger-Hüet anomaly, it is possible that the association of Sjögren's syndrome with this nuclear anomaly was not a mere coincidence but immunological defects found in the latter was responsible for the manifestation of the former.
一般社団法人日本血液学会の論文