新型布尼亚病毒感染六例临床分析

近年来,我国多个省份相继报道了一些以发热伴血小板减少为主要临床表现的感染性疾病病例,其中少数重症患者因多脏器损害而救治无效死亡。早些年曾经认为是嗜吞噬细胞无形体所致人粒细胞无形体病,后在一些疑似人粒细胞无形体病病例的样本中发现了一种新型布尼亚病毒,所致疾病现称为发热伴血小板减少综合征。我院近年来收治了6例确诊为新型布尼亚病毒感染所致发热伴血小板减少综合征患者,为提高对该疾病临床特征的认识,现将其临床特征总结如下。     

脓毒症相关性血小板减少症的危险因素及预后分析

目的研究脓毒症相关性血小板减少症的危险因素及对脓毒症患者预后的影响。方法将95例脓毒症患者根据血小板是否减少分成病例组（45例,血小板减少组）和对照组（50例,血小板未减少）。比较所有患者的一般情况、各实验室指标、28 d病死率及感染控制率,同时采用Logistic多因素回归法分析脓毒症相关性血小板减少患者死亡危险因素,采用Kaplan—Meier绘制两组患者的生存曲线。结果病例组患者中血液透析、肝素抗凝、解热镇痛药的使用,血培养阳性及发生弥漫性血管内凝血较对照组均明显偏高（P均〈0．05）,血小板最低值、C反应蛋白、降钙素原的比较差异均有统计学意义（P均〈0．05）。同时与对照组相比较,病例组患者病死率升高,感染控制率偏低（P均〈0．05）。Logistic多因素回归分析发现血小板减少〈3×10^9／L、血小板减少持续时间〉7 d、脓毒症休克、多器官功能衰竭是导致患者死亡的独立危险因素（P均〈0．05）。Kaplan—Meier生存分析结果显示病例组28 d累计生存率为44．4％,对照组为64．0％。 结论血小板减少〈3×10^9／L、血小板减少持续时间〉7 d、脓毒症休克、多器官功能衰竭是脓毒症相关性血小板减少的独立危险因素,且脓毒症患者出现血小板减少预示患者病情危重,预后差。     

江西省儿童血小板减少性紫癜发病率抽样调查

目的 了解江西省儿童血小板减少性紫癜的发病率.方法 对南昌市、宜春市和景德镇市所有县级及以上医院2007～2009年发病且年龄≤14岁的血小板减少性紫癜住院病例进行调查.结果 3市26个县区共调查77所医院,共搜索到549例儿童血小板减少性紫瘢病例;年平均发病率为7.20/10万,其中男性发病率为8.70/10万,女性为5.51/10万;0～4岁儿童发病率最高为15.75/10万,每年5、10月为发病最高峰.结论 江西省儿童血小板减少性紫癜发病率为7/10万左右,男性、婴幼儿易高发,无明显季节性.     

血小板生成素受体激动剂的临床应用及研究进展

血小板减少症为临床常见的疾病,可见于血液系统疾病与非血液系统疾病.随着对促血小板生成药物研究的深入,血小板生成素受体激动剂（TPORA）罗米司亭及艾曲泊帕的临床应用,可有效提高患者血小板计数,为治疗血小板减少症提供新的治疗方法.笔者拟就TPORA的作用机制、其在血液系统疾病及肝病相关的血小板减少症中的临床应用的进展进行综述.     

戈谢氏病伴血小板减少一例并文献复习

目的：探讨骨髓细胞形态学检查在戈谢氏病诊断中的价值。方法：采用瑞氏一吉姆萨染色法、糖原染色法对一例戈谢氏病伴血小板减少患者的骨髓片、血片进行化学染色,辅助骨髓基因及染色体检查,结合文献复习,提高对该病的认识。结果：瑞氏一吉姆萨染色发现骨髓片中的戈谢氏细胞,糖原染色该细胞呈阳性反应,确诊为戈谢氏病。结论：骨髓细胞形态学检查有助于戈谢氏病的早期诊断。     

浙江省德清县农村社区成人血小板减少调查

目的:了解德清农村社区成人居民血小板减少情况及可能影响因素。方法:采用流行病学现况研究设计,整群抽取浙江省德清县4个农村社区,面对面调查上述社区中所有18周～64周岁的社区户籍居民。血小板减少定义为<100×109/L。在Epidata3.1中文版建立数据库,SPSS11.0中进行数据分析。结果:在4479名调查对象中,血小板减少粗患病率为21.2%;按照2000年全国人口进行年龄性别标化的患病率为18.6%,患病率随着年龄的增加而增加(χ2趋势=14.43,P<0.001)。在非条件logistic回归分析中,年龄较大、女性、吸烟、家庭年人均收入中低水平可能增加血小板减少的风险,而BMI与之负相关。结论:农村社区成人居民血小板减少患病率较高,应加强监控。     

Histiocytic sarcoma of the spleen: case report of asymptomatic onset of thrombocytopenia and complex imaging features

Histiocytic sarcoma of the spleen, in which the malignant cells display morphologic and immunophenotypic features similar to those of mature tissue histiocytes, is a rare but potentially lethal condition that can remain asymptomatic or only mildly symptomatic for a long period of time. We studied a case of histiocytic sarcoma of the spleen in an 82-year-old woman with prolonged chronic thrombocytopenia that was non-responsive to steroid therapy. Ultrasonography, computed tomography, and magnetic resonance imaging showed a characteristically enlarged spleen and liver. Palliative irradiation therapy was clinically effective; however, disease progression proved lethal. Autopsy revealed the proliferation of tumor cells within the splenic sinus and the liver sinusoids, which displayed extreme hemophagocytosis and strong expression of the histiocytic markers CD68 (KP1 and PG-M1) and CD163. The postmortem diagnosis showed histiocytic sarcoma of the spleen with liver infiltration. This and previous reports indicate that early detection (facilitated by imaging and clinical features) and management may improve patient prognosis and survival. Histiocytic sarcoma of the spleen should be considered as a differential diagnosis in therapeutically unresponsive patients with chronic thrombocytopenia.     

病毒性肝炎血小板减少症影响因素的研究

目的探讨病毒性肝炎血小板减少症的发病机制.方法 84例病毒性肝炎患者和20名健康志愿者分为3组,A组(48例病毒性肝炎并血小板减少症患者)、B组(36例病毒性肝炎血小板正常患者)和C组(20名健康志愿者),分别采用酶联免疫吸附法、流式细胞术、腹部彩色B超检测3组血清血小板生成素(TPO)水平、血小板相关免疫球蛋白(PAIg)及其类别PAIgG、PAIgA、PAIgM水平、脾脏大小,采用骨髓穿刺术对其中74例行骨髓细胞学检查.结果血清TPO水平A组低于C组(P<0.01)和B组(P<0.05),严重肝病血清TPO水平与血小板数相关(r=0.374,P<00.01).PAIg、PAIgG水平A组明显高于B组(P<0.001)和C组(P<0.01),血小板数与PAIg水平呈负相关(r=0.446,P<0.01),血小板数与PAIgG水平亦呈负相关(r=-0.462,P<0.01).脾脏肿大发生率A组(77.1%)明显高于B组(47.2%,P<0.01),C组无脾脏肿大发生,血小板数与脾脏大小呈负相关(r=-0.5 81,P<0.01).74例骨髓象显示A组有4例呈骨髓抑制象改变,B组和C组无一例有上述改变.结论严重肝功能受损时血清TPO水平下降,与血小板数减少直接相关.PAIg介导的自身免疫机制在病毒性肝炎血小板减少症中可能起重要作用.脾脏肿大是引起病毒性肝炎血小板减少的因素.初步发现慢性肝病有骨髓抑制现象,可能成为引起病毒性肝炎血小板减少的因素之一.     

Transient blood plasmacytosis in parvovirus B19 infection: a report of two cases

Two patients with sickle cell disease were diagnosed with aplastic crisis caused by acute parvovirus B19 infection. One patient also developed thrombocytopenia associated with hemophagocytic histiocytosis in the bone marrow biopsy. Both patients developed transient blood plasmacytosis and hypocomplementemia soon after admission to the hospital. Transient blood plasmacytosis may be an immunological response to acute parvovirus B19 infection.     

Thrombotic thrombocytopenic purpura: a rare cause of thrombocytopenia in HIV-infected hemophiliacs

Thrombocytopenia is a common complication in human immunodeficiency virus (HIV)-infected hemophiliacs. The etiology is multifactorial and a majority of the patients with hemophilia exhibit a decreased platelet count within 10 years of seroconversion. Thrombocytopenia in these patients is associated with a high risk of bleeding and death. Thrombotic microangiopathy causing thrombocytopenia in HIV-infected hemophiliacs is extremely rare. We describe an HIV-infected hemophilic patient who presented with bleeding, renal insufficiency, and thrombocytopenia. Platelet transfusion resulted in deterioration of clinical condition. Examination of blood smears demonstrated a microangiopathic process. The patient responded well to plasmapheresis with normalization of platelet and renal function. Thrombotic thrombocytopenic purpura should be suspected in HIV-infected hemophiliacs who present with a new onset of thrombocytopenia and anemia as delay in treatment may result in fatal sequelae.