Xuemei Zhang, Yongshu Diao, Ling Zhang, Yingying Yang, Ping Fu
Division of Nephrology, West China Hospital, Sichuan University, Chengdu, China.
Iran Red Crescent Med J. 2016 Apr 25;18(10):e23272. doi: 10.5812/ircmj.23272v2. eCollection 2016 Oct.
We describe a female patient with extreme reactive thrombocytosis (RT) in anti-neutrophil cytoplasmic autoantibodies (ANCA)-associated crescentic glomerulonephritis (CGN) with immune complex deposits, which has never been reported before.
A female adolescent with symptoms of oliguria and gross hematuresis had serious renal function impairment (crescent formation and immune complex deposits in renal pathology examination with positive serum ANCA) and extreme thrombocytosis. We made a diagnosis of CGN and RT. After treatment with Prednisone, Cyclophosphamide, and plasmapheresis, the symptoms of oliguria and gross hematuresis were relieved remarkably and the serum creatinine and platelet count declined significantly.
The diagnosis of thrombocytosis is not easy in all cases. The coexistence of ANCA and the immune complex in CGN may cause a severe inflammatory state, leading to extreme RT. The roles that the immune complex and ANCA play on the effect of the platelet count and function in CGN need further research.
我们描述了一名患有抗中性粒细胞胞浆自身抗体（ANCA）相关性新月体性肾小球肾炎（CGN）且伴有免疫复合物沉积的女性患者出现极端反应性血小板增多症（RT），此前从未有过相关报道。
一名患有少尿和肉眼血尿症状的青春期女性，存在严重肾功能损害（肾脏病理检查显示有新月体形成和免疫复合物沉积，血清ANCA阳性）以及极端血小板增多症。我们诊断为CGN和RT。经泼尼松、环磷酰胺及血浆置换治疗后，少尿和肉眼血尿症状明显缓解，血清肌酐和血小板计数显著下降。
并非所有病例中血小板增多症的诊断都很容易。CGN中ANCA与免疫复合物的共存可能导致严重的炎症状态，进而引发极端RT。免疫复合物和ANCA在CGN中对血小板计数及功能的影响作用有待进一步研究。
