Nitschke M, Fink K, Pawlow-Handt S, Leeker A, Rob P M, Steinhoff J
Medizinische Klinik I, Medizinischen Universität Lübeck.
Dtsch Med Wochenschr. 1997 Oct 2;122(40):1213-6. doi: 10.1055/s-2008-1047750.
Two days after starting withdrawal treatment for alcohol and drug abuse a 56-year-old woman developed acute renal failure. The patient was in a poor general condition and disoriented as to time and place. She had uraemic oral fetor and leg oedema. She had previously fractured both arms 3 months before.
Biochemical tests indicated renal failure: creatine 1791 mumol/l, urea 51.7 mmol/l, potassium 5.3 mmol/l, phosphate 1.86 mmol/l. Serum protein electrophoresis suggested paraproteinaemia with M gradients in the gamma-fraction. Immune fixation electrophoresis demonstrated monoclonal IgM gammopathy of kappa-type (IgM 44.1 g/l). Haemoglobin level was reduced to 66 g/l. Bone marrow biopsy showed replacement of normal haematopoiesis by highly atypical plasma cells (> 30% of cell population). Magnetic resonance imaging revealed diffuse changes in the pelvis and vertebrae suggesting plasmacytoma, confirming the diagnosis of IgM plasmacytoma of kappa-type.
Focal neurological symptoms (e.g. intermittent anisocoria and visual disturbances) suggested a hyperviscosity syndrome, although the serum protein level was nearly normal. Plasma viscosity was 2.2 mPas (normal range 1.2-1.38 mPas), lowered to 1.5 mPas by plasmapheresis, after which the neuropsychiatric symptoms improved. Chemotherapy for the plasmacytoma in stage IIIB was initiated (VAD scheme) and dialysis became necessary for terminal renal failure.
Due to the raised level of IgM protein level and its high molecular size a hyperviscosity syndrome with paraproteinemic coma may occur, even though total plasma protein is nearly normal.
一名56岁女性在开始戒酒及戒毒治疗两天后出现急性肾衰竭。患者一般状况较差,存在时间和地点定向障碍。她有尿毒症口臭和腿部水肿。3个月前她曾双臂骨折。
生化检查提示肾衰竭:肌酐1791μmol/L,尿素51.7mmol/L,钾5.3mmol/L,磷酸盐1.86mmol/L。血清蛋白电泳提示副蛋白血症,γ区有M峰。免疫固定电泳显示κ型单克隆IgM丙种球蛋白病(IgM 44.1g/L)。血红蛋白水平降至66g/L。骨髓活检显示正常造血被高度不典型浆细胞替代(占细胞总数>30%)。磁共振成像显示骨盆和椎体弥漫性改变,提示浆细胞瘤,确诊为κ型IgM浆细胞瘤。
尽管血清蛋白水平接近正常,但局灶性神经症状(如间歇性瞳孔不等大和视觉障碍)提示高黏滞综合征。血浆黏度为2.2mPas(正常范围1.2 - 1.38mPas),通过血浆置换降至1.5mPas,之后神经精神症状改善。开始对IIIB期浆细胞瘤进行化疗(VAD方案),终末期肾衰竭需要透析。
尽管总血浆蛋白接近正常,但由于IgM蛋白水平升高及其高分子量,可能会发生伴有副蛋白血症昏迷的高黏滞综合征。