Plasma cell dyscrasiaおよび内分泌学的異常を伴う多発性神経炎の一例—Plasmapheresisの試み

POLYNEUROPATHY ASSOCIATED WITH PLASMA CELL DYSCRASIA AND ENDOCRINOLOGICAL ABNORMALITIES:A TRIAL OF PLASMAPHERESIS

　抄録　1gG　λ型M蛋白を分泌する形質細胞腫に伴う多発神経炎に対して血漿交換が試みられた。患者は27歳の男性で，7ヵ月にわたる慢性進行性多発神経炎による完全四肢麻痺の状態で入院し，浮腫，色素沈管，いくつかの内分泌学的異常を示し，入院直後に呼吸不全におちいった。Prednisolone 120 mgを投与しながら，1回70％を2日おきに3回血漿を交換したが，M蛋白の低下にもかかわらず短期的には臨床効果は得られなかった。しかし，腫瘍への放射線照射によって，迅速な臨床的改善を得た。しかし，M蛋白の推移と臨床的改善との間には良い相関は認められなかった。慢性進行性末梢神経障害に対する血漿交換の効果判定には，非常に困難な点があるが，少なくとも，形質細胞からの何らかの液性因子が本疾患において関与しており，その分泌は，我々が行なった程度の血漿交換では追いつけないが，腫瘍への放射線照射によって容易に抑えられることが予想された。したがって，腫瘍局在の明らかで重篤な症例には，まず放射線療法が第一に選択されるべきと考えられる。本症例においては，さらにホルモン学的検索および考察も若干加えた。

Plasmapheresis and irradiation of the tumor were performed in conjunction with prednisolone therapy to a severe case of polyneuropathy asso-ciated with a solitary sclerotic secreting myeloma and endocrinological abnormalities.

The patient was 27-year-old male with progres-sive history of polyneuropathy of seven months duration. On admission, he was quadriplegic with cranial nerve involvements. Pitting type of gene-ralized edema, skin pigmentation and sensory im-pairment were also present. Respiratory failure occured soon after the admission.

Plasmapheresis lowered the level of M protein but failed to improve clinical course in the short term. On the other hand, prompt amelioration of clinical symptoms followed the irradiation of the tumor.

It is difficult to prove clinical effects of plasma-pheresis upon symptoms of chronic polyneuropa-thy. It is suggested that some humoral factor of myeloma might attribute to this syndrome and se-creted at higher rate than our plasmapheresis but easily suppressed by the irradiation of the tumor. We suppose that radiation therapy could be the treatment of the first choice for such a severe case like ours. However, further experiences of plasma-pheresis of this syndrome arc necessary to de-termine the effect of plasmapheresis. Some other etiological aspects are also discussed.