第466例——肌无力、发热、视物模糊、血三系减少

The 466th case:myasthenia,fever,blurred vision and pancytopenia

患者女性,45岁。因双上肢无力、发热、视物模糊收入北京协和医院风湿免疫科。诊断系统性红斑狼疮(SLE)合并原发性胆汁性肝硬化,肾脏、视网膜、血液系统、肌肉受累,合并严重肺部感染、呼吸衰竭。予糖皮质激素、熊去氧胆酸及抗生素、呼吸支持治疗后短暂好转,再次出现发热,同时血小板下降至30×10^9/L,血红蛋白下降至78g/L,纤维蛋白原下降至<1.5g/L,铁蛋白升高至1640ng/ml,自然杀伤细胞数8/μl,自然杀伤细胞活性2%(参考值9.5%~23.5%),考虑噬血细胞性淋巴组织细胞增生症(HLH)。患者巨细胞病毒pp65抗原检测,13个阳性细胞/2×10^5 WBC,考虑HLH由巨细胞病毒感染所致可能性大,加用更昔洛韦250mg、2次/d静脉点滴治疗,患者体温逐渐降至36.5℃,血小板升至229×10^9/L,血红蛋白升至94g/L,纤维蛋白原升至3.26g/L。SLE治疗过程中出现原发病不能解释的危急征象时,需警惕感染、HLH、血栓性血小板减少性紫癜等严重并发症。

A 45-year-old woman was admitted to the Department of Rheumatology and Immunology, Peking Union Medical College Hospital, due to weakness of the upper limbs, fever, and blurred vision. She was clinically diagnosed as systemic lupus erythematosus overlapped primary biliary cirrhosis, with renal, retinal, hematological and musculoskeletal involvement, combined with severe pulmonary infection and respiratory failure. Treated with glucocorticoids, ursodeoxycholic acid, antibiotics and respiratory support, the patient got better. A couple of days later, her fever recurred and platelets count dropped to 30×109/L, hemoglobin to 78 g/L, fibrinogen to<1.5 g/L, ferritin to 1 640 ng/ml, natural killer (NK) cell count to 8/μl, the activity of NK cells 2% (reference value 9.5%-23.5%), considering the occurrence of hemophagocytic lymphohistiocytosis (HLH). Cytomegalovirus pp65 antigenemia test: 13 positive cells/2×105 WBC. Considered the possibility of HLH caused by cytomegalovirus infection and treated by 250 mg ganciclovir intravenous drip twice a day for a full course. The temperature of the patient was gradually reduced to 36.5℃, the count of platelets were increased to 229×109/L, the hemoglobin was increased to 94 g/L, and the fibrinogen was increased to 3.26 g/L. When there were unexplained critical signs of the primary disease during systemic lupus erythematosus treatment, severe complications such as infection, HLH, thrombotic thrombocytopenic purpura should be taken into account.