老年原发免疫性血小板减少症患者治疗前后T淋巴细胞亚群的临床研究

目的 检测老年原发免疫性血小板减少症(ITP)患者治疗前后T淋巴细胞亚群的动态变化, 探讨其在ITP发生发展中的作用。方法 采用流式细胞术测定ITP患者治疗前后及正常对照组外周血T淋巴细胞亚群的水平。结果 ITP患者治疗前后T淋巴细胞绝对值、CD3⁺、CD4⁺、CD8⁺、CD4⁺CD25⁺T淋巴细胞比例及CD4⁺/CD8⁺比值分别为(0.83±0.16)vs(1.74±0.36)、(71.71±1.07)% vs(72.69±1.35)%、(41.78±0.71)% vs(42.46±1.20)%、(29.67±0.97)% vs(28.56±1.75)%、(8.76±0.56)% vs(9.39±1.26)%、(1.42±0.07)vs(1.49±0.13), CD8⁺T淋巴细胞比例治疗后显著降低, 其余均显著升高, 差异有统计学意义(P<0.05)。结论 T淋巴细胞亚群的异常改变, 破坏自身免疫, 与病情相关, 可指导临床治疗, 并作为评估预后的参考指标。     

Heparin-induced thrombocytopenia with multiple organized thrombi accompanied by unusual cholesterin deposition: Autopsy case after long-term follow up

Heparin-induced thrombocytopenia (HIT) is characterized by a reduction in the platelet count and systemic thromboembolism during heparin therapy. Herein is reported a case of HIT with characteristic thrombus formation. A 68-year-old man who had been treated for hypertension for 27 years suffered a brain infarction and was treated with heparin. After this treatment, other new infarctions occurred in multiple organs. Because serum antibodies against heparin/PF4 complex were detected, he was diagnosed as having HIT, and warfarin and argatroban were administered instead of heparin. He died, however, 119 days after the first onset. At autopsy infarction due to organized thrombi with cholesterin deposition in multiple organs were found, similar to usual atherosclerotic emboli, but different to them with regard to clinical course and distribution of thrombi. This case in which organization and frequent cholesterin deposition were found in thromboembolized lesions of multiple organs after relatively long-term follow up, is unusual. The findings suggest that HIT accompanied by marked hypercholesterolemia of long duration contributes to a characteristic form of thromboembolism that needs careful management.     

Congenital Rosai–Dorfman disease presenting with anemia,thrombocytopenia, and hepatomegaly

Rosai–Dorfman disease (RDD) is a rare entity of non‐Langerhans cell histiocytoses (non‐LCH) which usually presents with bilateral painless cervical lymphadenopathy. We describe a neonate with RDD who presented with anemia, thrombocytopenia and hepatomegaly. He recovered spontaneously with conservative management. This represents an atypical presentation of RDD. Conservative management with close monitoring can be adopted for some with systemic involvement. Pediatr Blood Cancer 2009;52:415–417. © 2008 Wiley‐Liss, Inc.     

Refractory autoimmune thrombocytopenic purpura: responses to treatment with a recombinant antibody to lymphocyte membrane antigen CD20 (rituximab)

"Refractory" autoimmune thrombocytopenia represents a life-threatening condition, having failed to respond to a variety of therapeutic measures. We report a series of cases, all failing splenectomy and multiple therapeutic programs, including, in two patients, marrow transplant. Five of the six cases reported responded to a recombinant antibody to the lymphocyte membrane antigen CD20 (rituximab), an agent commonly employed in the treatment of non-Hodgkin's lymphoma. Our experiences over a period of 4 years are documented. The results support the use of this product, rituximab, in the treatment of patients with autoimmune thrombocytopenia who have not attained a hemostatically effective platelet count following splenectomy and require a continuing therapeutic management program.     

重组人白介素-11联合环孢素治疗糖皮质激素无效ITP的临床研究

目的 探讨重组人白介素-11(rhIL-11)联合环孢素治疗糖皮质激素无效的原发免疫性血小板减少症(ITP)的远期临床疗效及其安全性。方法 选取糖皮质激素治疗无效的ITP患者60例,患者随机分为治疗组(40例)和对照组(20例)。治疗组应用rhIL-11和环孢素治疗,对照组长春新碱和静脉注射用免疫球蛋白治疗,分别在治疗前、后检测两组血小板计数,观察两组临床疗效和不良反应。结果 治疗组第1个月、3个月、5个月、12个月末的有效率分别为82.5%、80.0%、72.5%和52.5%,对照组的有效率分别为55.0%、50.0%、40.0%和25.0%,治疗组均优于对照组,差异有统计学意义(P<0.05)。治疗组、对照组不良反应轻微,大多数患者耐受良好。结论 rhIL-11联合环孢素治疗糖皮质激素无效的ITP的中远期疗效满意,安全性良好,可作为理想的二线方案。     

Steroid pulse therapy in patients with encephalopathy associated with severe fever with thrombocytopenia syndrome

Severe fever with thrombocytopenia syndrome (SFTS) is a tick-borne infectious disease caused by the SFTS virus (SFTSV). Clinical symptoms of SFTS often involve encephalopathy and other central neurological symptoms, particularly in seriously ill patients; however, pathogenesis of encephalopathy by SFTSV is largely unknown. Herein, we present case reports of three patients with SFTS, complicated by encephalopathy, admitted to Tokushima University hospital: one patient was a 63-year-old man, while the other two were 83- and 86-year-old women. All of them developed disturbance of consciousness around the 7th day post onset of fever. After methylprednisolone pulse therapy of 500 mg/day, all of them recovered without any neurological sequelae. SFTSV genome was not detected in the cerebrospinal fluid of 2 out of the 3 patients that were available for examination. In these patients, disturbance of consciousness seemed to be an indirect effect of the cytokine storm triggered by SFTSV infection. We propose that short-term glucocorticoid therapy might be beneficial in the treatment of encephalopathy during early phase of SFTSV infection.     

基于上转化发光免疫层析技术建立发热伴血小板减少综合征病毒总抗体快速检测方法

目的基于上转化发光(UPT)免疫层析技术,建立发热伴血小板减少综合征病毒(SFTSV)总抗体的现场快速检测方法。方法将SFTSV重组NP蛋白与上转化发光颗粒(UCP)偶联,制备UCP-NP免疫层析试纸条,评价该试纸条检测SFTSV总抗体的灵敏性、特异性和稳定性,并检测SFTSV血清254份,与酶联免疫法(ELISA)比较。结果该方法可在15min内完成SFTSV总抗体检测,可检测1∶500稀释度的SFTSV阳性血清,与其他出血热病毒无交叉反应,加样14d内稳定性较高。UPT免疫层析法与ELISA法检测临床血清样品一致性极高(Kappa=0.967),约登指数为0.973。结论建立了基于UPT免疫层析技术的SFTSV总抗体快速检测方法,该方法灵敏、特异,且操作简便、快速,结果稳定,适合在基层门诊和体检现场推广。