艾曲波帕治疗儿童造血干细胞移植后血小板减少症24例分析

目的评价艾曲波帕治疗儿童造血干细胞移植(HSCT)后血小板减少症的疗效与安全性。方法回顾性分析2018年8月1日至2019年4月1日于中山大学孙逸仙纪念医院儿科接受HSCT后发生血小板减少症并采用艾曲波帕治疗的24例患儿的临床资料,评估治疗有效率及不良反应,根据造血干细胞来源分为脐带血组和外周干细胞组,根据疾病类型分为恶性病组和非恶性病组,分析各组间临床疗效。组间比较采用秩和检验。结果 24例患儿中男15例、女9例,艾曲波帕用药时的年龄7.7(2.6~13.7)岁,接受艾曲波帕治疗的时间为移植后第27.5(8.0~125.0) d,使用后完全有效的时间为23.5(6.0~83.0) d,用药疗程为36.5(8.0~90.0) d,艾曲波帕总剂量为1 400(200~5 900)mg,完全有效率92%(22/24),未发生艾曲波帕相关不良反应。脐带血干细胞移植(16例)的患儿使用艾曲波帕的疗程及总剂量均明显低于外周干细胞移植组(8例)[24.5(8.0~81.0)比65.5(35.0~90.0)d,900.0(200.0~3 850.0)比2 862.5 (1 175.0~5 900.0) mg,Z=-3.004、-2.604,P=0.002、0.007],而达到完全有效的时间及停药2周后血小板计数、随访终点血小板计数的差异均无统计学意义(均P>0.05)。与恶性病患儿(12例)相比,非恶性病患儿(12例)用药后至获得完全有效的时间、用药疗程、总剂量及停药2周后血小板计数、随访终点血小板计数的差异均无统计学意义(均P>0.05)。结论艾曲波帕用于儿童HSCT后血小板减少症有一定疗效,安全性高,尤其在脐带血移植中可能更有优势。     

新型布尼亚病毒感染患者凝血功能异常及其临床意义

目的探讨新型布尼亚病毒感染后患者发热伴血小板减少症状(SFTS)发病期和恢复期的凝血和血栓相关指标及其临床意义。方法选择2020年4月12日至8月12日在安徽医科大学第一附属医院收治的新型布尼亚病毒感染后SFTS患者,其中SFTS发病期36例,SFTS恢复期18例,并招募36名健康人作为健康对照组,采集血浆标本。回顾性分析血浆凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)、纤维蛋白原含量(FIB)、血浆凝血酶时间(TT)、抗凝血酶-Ⅲ(AT-Ⅲ)、纤维蛋白降解产物(FDP)、D-二聚体(D-D)、血栓调节蛋白(TM)、凝血酶-抗凝血酶复合物(TAT)、纤溶酶-α2纤溶酶抑制物复合物(PIC)、组织型纤溶酶原激活剂-抑制剂1复合物(t-PAIC)浓度。统计比较3组间上述指标的差异。结果与健康对照组比较,SFTS发病组PT[12.5(12.1,13.6)s比10.8(10.5,11.5)s,P<0.05]较长,但仍在正常参考值范围内(14.0~21.0 s);APTT[49.1(42.0,58.2)s比28.5(26.6,30.4)s,P<0.05]较长;FDP[6.07(2.67,8.64)μg/ml比1.00(0.80,1.87)μg/ml]和D-D[2.27(1.04,2.98)μg/ml比0.30(0.21,0.47)μg/ml]较高(P均<0.001);血浆TAT[16.05(8.05,26.58)ng/ml比3.55(2.60,4.85)ng/ml]和PIC浓度[4.44(2.52,5.54)μg/ml比0.84(0.60,1.35)μg/ml]较高(P均<0.001);TM[(19.41±8.29)TU/ml比(9.33±1.89)TU/ml]和t-PAIC浓度[(37.52±21.10)ng/ml比(7.06±3.37)ng/ml]较高(P均<0.001)。SFTS恢复组患者血浆中TAT浓度为9.10(3.95,18.40)ng/ml,仍高于正常参考范围(<4 ng/ml),PIC浓度低于SFTS发病组[1.91(1.45,2.93)μg/ml比4.44(2.52,5.54)μg/ml,P<0.05],TM和t-PAIC低于SFTS发病组(P均<0.05)。结论SFTS患者发病期机体凝血系统激活,血管内皮受到损伤;恢复期血管内皮损伤情况减轻,但机体依然存在一定程度的凝血系统障碍,提示当体内病毒清除后,仍需继续监测凝血指标。     

A case of idiopathic portal hypertension associated with rheumatoid arthritis

Abstract

A 53-year-old woman who had been diagnosed with rheumatoid arthritis was found to have thrombocytopenia, splenomegaly, and gastric varices. She was diagnosed as having idiopathic portal hypertension on the basis of liver biopsy and angiography. Treatment with prednisolone was not sufficiently effective for thrombocytopenia. After transabdominal devascularization with splenectomy, thrombocytopenia subsided and gastric varices disappeared. In this case, the autoimmune mechanism as well as hypersplenism was suspected of being involved in the mechanism of thrombocytopenia.     

Delayed thrombocytopenia following abciximab therapy

Inhibitors of glycoprotein (GP) IIb/IIIa are currently approved for the treatment of acute coronary syndromes and during performance of percutaneous coronary interventions (PCIs). More than 500 000 patients annually undergo PCIs in the USA alone. Of these, 35% are receiving GPIIb/IIIa inhibitors. Currently, three different intravenous GPIIb/IIIa inhibitors are commercially available. Profound thrombocytopenia occurs almost exclusively with abciximab. Usually thrombocytopenia develops within 24 hours following abciximab administration. This paper describes three patients who developed delayed profound thrombocytopenia, occurring five days following abciximab therapy. These cases of thrombocytopenia were self-limited and reversible. Absence of serious bleeding complications was noted. The pathophysiology, differential diagnosis, natural history and management of the coronary patients with abciximab-induced thrombocytopenia are discussed.     

外周血淋巴细胞CD16＋CD56表达率对血小板减少性疾病鉴别诊断的价值

目的探讨外周血淋巴细胞CD16＋CD56表达率对不同血小板减少性疾病鉴别诊断的价值。方法应用流式细胞术检N28例正常对照者、47例特发性血小板减少性紫癜（ITP）患者、16例再生障碍性贫血（AA）患者、18例骨髓增生异常综合征（MDS）患者、13例急性淋巴细胞白血病（ALL）患者、37例急性非淋巴细胞白血病（ANLL）患者、11例慢性粒细胞白血病（CML）患者、6例慢性淋巴细胞白血病（CLL）患者和12例自身免疫性溶血性贫血（AIHA）患者外周血淋巴细胞CD16＋CD56表达率。结果ITP组外周血淋巴细胞CD16＋CD56表达率低于正常对照组（P〈0．05）;AA组低于正常对照组（P〈0．05）,高于MDS（P〈0．05）;白血病组较正常对照组明显降低（P〈0．05）,ALL组、ANLL组、CML组、CLL组之间无明显差异,白血病患者外周血淋巴细胞CD16＋CD56表达率随病情发展、复发而降低,病情控制缓解而上升;AIHA组与正常对照组相比无明显差异。结论外周血淋巴细胞CD16＋CD56表达率的检测对鉴别血小板减少性疾病有重要意义,对患者的疗效及预后判断有一定的参考价值。     

Campylobacter Jejuni and Cytopenias

Background

Leukopenia and thrombocytopenia in a febrile patient are not uncommon and may be a diagnostic clue in patients without an alternative explanation for cytopenias. This has not been reported in Campylobacter jejuni infections.

Methods

A healthy patient with fever, rigors, and an acute diarrheal illness was noted to have a white blood cell count of 2.65 × 10⁹/L and platelet level of 125 × 10⁹/L. Retrospective chart review of all adult C. jejuni stool-positive cases admitted over 1 year revealed leukopenia in 6 of 20 (30%), thrombocytopenia in 5 of 20 (25%), and both in 1 of 20 (5%).

Results

Cytopenias were mild, transient, and not associated with prolonged hospital stay or complications.

Conclusions

Acute C. jejuni infections should be added to the differential diagnosis of acute febrile illnesses that may be associated with leukopenia or thrombocytopenia. Cytopenias can be an important diagnostic clue in febrile illnesses, and their differential is presented.     

Familial, atypical hemolytic-uremic syndrome in a premature infant

The hemolytic-uremic syndrome (HUS) typically presents in toddlers or older children after an episode of bloody diarrhea caused by Escherichia coli 0157:H7. However, numerous ’’atypical’’ presentations have been described, including familial cases. Here we describe what we believe to be the first report of familial HUS in a premature infant during the neonatal period. Received December 19, 1997; received in revised form April 13, 1998; accepted April 14, 1998     

抗人TPO单克隆抗体的制备

目的：制备抗人血小板生成素（hTPO）单克隆抗体，用于建立ELISA方法和检测TPO的表达。方法：用基因重组的hTPO免疫Balb／c小鼠，常规法做细胞融合，用ELISA法筛选出阳性杂交瘤细胞株。结果：获得一杂交瘤细胞株（46-6），其免疫球蛋白亚类为IgG1经ELISA和免疫印迹技术证明，此单抗能特异识别TPO。结论：46－6单克隆抗体（McAb）特异性强。用其建立的夹心ELISA检测TPO的方法线性好，灵敏度可达50ng／L。