骨髓巨核细胞数及血小板4项参数在特发性血小板减少性紫癜患者中价值

目的探讨特发性血小板减少性紫癜(idiopathic thrombo-cytopenic purpura,ITP)患者初诊时血小板4项参数及骨髓涂片巨核细胞计数的价值。方法分析200例ITP患者(ITP组)及60例骨髓检验正常者(正常对照组)血小板4项参数关系,比较治疗前后血小板4项参数变化并观察治疗过程中血小板上升速度及骨髓涂片巨核细胞计数与治疗结果之间的关系。结果 ITP组治疗后巨核细胞数低于治疗前,(69.5±14.6)个/片vs(178.8±54.1)个/片(P<0.01),产巨核细胞比例、血小板计数(PLT)、血小板压积(PCT)、血小板平均体积(MPV)、血小板分布宽度(PDW)高于治疗前,分别为(79.4±31.2)%vs(5.2±2.7)%、(86.9±52.3)×109/L vs(37.2±13.7)×109/L、(3.10±0.63)%vs(1.40±0.35)%、(9.6±1.7)fl vs(9.0±1.7)fl、(19.2±1.5)%vs(17.3±1.3)%(P<0.05或<0.01),但巨核细胞数、产巨核细胞比例、PCT、MPV和PDW仍高于正常对照组,PLT则低于正常对照组。ITP患者初诊时PLT不同水平间疗效差异无统计学意义(P>0.05),而初诊时不同巨核细胞数组间疗效差异有统计学意义(P<0.01)。ITP患者初诊时外周PLT与疗效无关,治疗2周内患者PLT超过100×109/L其基本治愈率较高(96.0%);每单位涂片(1.5 cm×3 cm)巨核细胞计数>100个的患者治愈率达85.7%,初诊时骨髓涂片巨核细胞计数越多,患者的治疗效果越好。结论观察血小板参数对骨髓产血小板功能及治疗疗效观察有重要意义。ITP患者初诊时进行骨髓检查,并计数巨核细胞数,对诊断及治疗效果的判定均是必要的。在治疗过程中及时复查血像,观察血小板上升速度,对预后的判定亦有重要价值。     

探讨免疫性血小板减少性紫癜血小板计数与骨髓巨核细胞的关系

目的：探讨免疫性血小板减少性紫癜（ITP）血小板计数与骨髓巨核细胞数及分类关系。方法：对145例ITP患者根据外周血血小板计数进行分组：Ⅰ组53例（血小板计数〈20×10^9／L）,Ⅱ组49例（血小板计数20×10^9／L）,Ⅲ组43例（血小板数50-80×10^9／L）,各组患者分别行骨髓穿刺、涂片、染色后,用光学显微镜计数全片巨核细胞数,再根据巨核细胞形态特点进行分类,每例患者分50个巨核细胞。结果：Ⅰ、Ⅱ、Ⅲ组巨核细胞总数与对照组比较差异有显著性（P〈0．01）;巨核细胞分类中,除原始型巨核细胞Ⅱ、Ⅲ组相比无统计学意义（P〉0．05）外,幼稚、颗粒、产血小板及裸核型巨核细胞三组之间两两相比较差异均有显著性（P〈0．01）。结论：在ITP时,外周血血小板数与骨髓中巨核细胞成熟情况有密切联系,骨髓中产板型巨核细胞越多,外周血小板计数就越多。     

特发性血小板减少性紫癜患者CD138表达情况及其意义

目的:探讨CD138细胞与特发性血小板减少性紫癜(ITP)惠者预后,及其与相关临床指标的关系.方法:用流式细胞术检测100例初诊ITP患者和20例正常对照外周血CD138细胞表达率.随访6个月后,将ITP患者分为急性型与慢性型二组,比较初诊时外周血CD138细胞表达率,并分析CD138细胞表达率与就诊时间、初诊时血小板计数、血小板相关抗体、骨髓巨核细胞计数、治疗反应的关系.结果:外周血CD138细胞表达率,急性型组与对照组无差异(P>0.05),慢性型组明显高于对照组,差异有统计学意义(P<0.05);慢性型患者外周血CD138细胞表达率与初诊时血小板计数、血小板相关抗体无相关(P>0.05),与就诊时间、骨髓巨核细胞计数、治疗反应相关(P<0.05).结论:CD138细胞在慢性型ITP患者外周血中高表达,可作为早期判断ITP惠者预后的重要指标之一.     

115例特发性血小板减少性紫癜骨髓活检巨核细胞计数与临床疗效关系的研究

本研究首次评价特发性血小板减少性紫癜骨髓涂片及活检巨核细胞计数与临床疗效关系。按临床类型、血小板计数、骨髓涂片及活检巨核细胞计数115例成人ITP患者疗效。结果表明:①急性ITP患者疗效优于慢性ITP患者,2组近期治疗有效率分别为86.6%及60.4%(p<0.01),而远期治疗有效率分别为82.5%及68.9%(p<0.05)。②发病时不同血小板计数的ITP患者,他们的近期及远期疗效无显著差异(p>0.05)。③按发病时骨髓涂片巨核细胞计数进行分组,即涂片巨核细胞计数小于7个/4.5cm2组、7-35个/4.5cm2组、大于35个/4.5cm2组,这3组的近期治疗有效分别率为53.3%、73.8%及86.2%,这3组间相比较有显著差异(p<0.01),其远期治疗有效率分别为42.8%、84.6%及85.5%,而后2组治疗有效率相比较无显著差异(p>0.05),但后2组分别同第1组比较均有显著差异(p<0.01)。④按发病时骨髓组织切片巨核细胞计数进行分组:即切片巨核细胞计数小于8个/mm2组、8-15个/mm2组和大于15个/mm2组,这3组近期治疗有效率分别为53.8%、85.0%及90.3%,后2组治疗...     

布氏杆菌病急性期患者血小板检测25例分析

目的:检测25例布氏杆菌病急性期患者的外周血血小板计数,观察其变化特点.方法:提取患者肘静脉血,检测血小板计数,对初诊时考虑为原发性血小板减少性紫癜或有出血表现的患者予检测血小板相关抗体,并用骨髓涂片染色法对巨核细胞进行计数分类.结果:25例患者中,血小板减少9例(36%),其中5例进行血小板相关抗体检测,血小板相关抗体IgG均升高,血小板相关抗体IgA升高2例.骨髓象示巨核细胞数量正常,颗粒型巨核细胞增多,产血小板型巨核细胞减少.予肾上腺皮质激素治疗后5～8 d血小板计数恢复正常.结论:血小板的变化有助于判断疗效,当血小板计数小于50×109/L或有出血表现时可酌情应用肾上腺皮质激素.     

直接抗球蛋白试验阴性的Evans''''综合征含有自身抗Jk‘抗体

少数自身免疫性溶血性贫血(AIHA)伴有特发性血小板减少性紫癜(ITP),称之为Evans′综合征。通常AIHA直接抗球蛋白试验(DAT)阳性,阴性者少见。作者报告1例DAT阴性的Evans′综合征含有自身抗Jk~a抗体。抗Jk~a作为自身抗体出现罕见。患者为高加索人,34岁,因呼吸困难2天入院。既往健康,无输血史。体检发现苍白及中度黄疸。血红蛋白4.9g/dl,白细胞10.5×10~9/L,血小板496×10~9/L,网织红细胞6.8%(绝对值115×10~9/L),血片示球形红细胞增多,骨髓红系增生,巨核细胞正常,诊断为AIHA。经输注9个单位的压积红细胞及强的松龙治疗后,血红蛋白升至11.8g/dl,但血小板降至13×10~9/L,骨髓中巨核细胞增多,此时已发生     

骨髓巨核细胞和有核细胞计数的实验报告

目的 为了给确诊血液病及观察其疗效、预后,或判断骨髓移植造血功能恢复提供具有重要意义的骨髓巨核细胞数和有核细胞数定量数据,使该二项检验能为临床提供更准确、重复性更好的信息。方法 采用抗凝骨髓液、定量骨髓涂片、检查全涂片的巨核细胞以达到定量计数巨核细胞,并以传统每张涂片法作对照;以20倍稀释后用血细胞计数池计数的方法,达到定量计数骨髓有核细胞。结果 传统的每张涂片法38张涂片计数结果巨核细胞为299.97±97.27个/张(?±s),变异系数为65.8％;新推荐的骨髓巨核细胞定量计数法34张涂片结果为64.72±17.09/μl(?±s),变异系数为26.4％。114例骨髓有核细胞定量计数结果介于(6.0～456.0)×10~9/L之间((?97.3×10~9/L);三份骨髓有核细胞高、中、低值标本反复分别定量计数30次,变异系数分别为6.1％,6.1％和9.8％。结论 该研究推荐的骨髓巨核细胞和骨髓有核细胞定量计数法,与传统的每张涂片法或成熟红细胞与有核细胞比值及低倍镜估计法比较,具有方法学设计科学合理、重复性好,可提供具体而确切的数据,便于临床动态分析比较;同时还根据定量计数有核细胞的骨髓涂片,提出了一个高倍镜判断有核细胞增生程度的标准,可供必要时选用。     

获得性低巨核细胞性血小板减少性紫癜17例分析

获得性低巨核细胞性血小板减少性紫癜(AATP)近年来逐渐有人报告。其主要特点是骨髓巨核细胞减少或缺如伴血小板减少,临床上以出血为主要表现。我们遇到17例,现报告如下。 1 临床资料 1.1 一般资料:本组17例均为我院住院患者,其中男7例,女10例,年龄12至62岁。诊断标准为骨髓涂片单位面积(1.5×3.0cm)巨核细胞少于7只,排除其它疾病及骨髓稀释。本组初诊时全部误诊,其中诊为原发性血小板减少性紫癜(ITP)9例,急性再生障碍性贫血5例,慢性再障2例,白血病1例。发病前有“上感”5例,1例误服桐油史,1例有     

静脉输注胎盘丙种球蛋白治疗免疫性血小板减少性紫癜

免疫性血小板减少性紫癜(ITP)诊断标准:①多次检查血小板计数减少<55×10~9/升;②脾不大;③骨髓中巨核细胞数增多或正常,有血小板成熟障碍现象,④排除其他血液病。具备4项即可诊断。     

特发性血小板减少性紫癜患者巨核细胞和血小板参数的探讨

目的:探讨骨髓巨核细胞与外周血血小板参数的变化在特发性血小板减少性紫癜(ITP)鉴别诊断中的价值。方法:研究设ITP患者组(46例)、非ITP患者组(45例)和健康人对照组(48例),分别检测ITP和非ITP组患者骨髓中巨核细胞及外周血血小板参数及健康对照组外周血血小板参数;采用t检测进行统计学分析。结果:与非ITP组相比,ITP组患者骨髓中巨核细胞数量明显增加,其中颗粒巨、幼巨核细胞明显增加,产板巨核细胞明显减少,差异有统计学意义(P0.01),而外周血血小板各参数2组间无差异(P0.05)。ITP组和健康对照组相比,外周血小板数量和压积均明显降低,有统计学意义(P0.01)。结论:骨髓巨核细胞数目、分类及外周血血小板各参数,对ITP的鉴别诊断具有一定的参考价值。     

Stimulation of thrombopoiesis in mice by human recombinant interleukin 6.

To date, testing of various cytokines for the stimulation of blood cell production has not demonstrated a consistent effect on peripheral platelet levels. In this report, we provide evidence that human recombinant IL-6 increased platelet production in mice, as measured by both peripheral platelet levels and [75Se]selenomethionine (75SeM) incorporation into newly forming platelets. Peripheral white blood cell counts also were increased, but only to a modest extent, and hematocrit values were unchanged. A dose-response relationship between the amount of IL-6 administered and platelet count, 75SeM incorporation, and white blood cell count was demonstrated. Detectable megakaryocyte and granulocyte-macrophage colony-forming cells in mice that had received IL-6 also were increased in both bone marrow and spleen. These results demonstrate the ability of a purified, recombinant protein to stimulate platelet production in vivo.     

Leukocytosis: basics of clinical assessment

Leukocytosis, a common laboratory finding, is most often due to relatively benign conditions (infections or inflammatory processes). Much less common but more serious causes include primary bone marrow disorders. The normal reaction of bone marrow to infection or inflammation leads to an increase in the number of white blood cells, predominantly polymorphonuclear leukocytes and less mature cell forms (the "left shift"). Physical stress (e.g., from seizures, anesthesia or overexertion) and emotional stress can also elevate white blood cell counts. Medications commonly associated with leukocytosis include corticosteroids, lithium and beta agonists. Increased eosinophil or basophil counts, resulting from a variety of infections, allergic reactions and other causes, can lead to leukocytosis in some patients. Primary bone marrow disorders should be suspected in patients who present with extremely elevated white blood cell counts or concurrent abnormalities in red blood cell or platelet counts. Weight loss, bleeding or bruising, liver, spleen or lymph node enlargement, and immunosuppression also increase suspicion for a marrow disorder. The most common bone marrow disorders can be grouped into acute leukemias, chronic leukemias and myeloproliferative disorders. Patients with an acute leukemia are more likely to be ill at presentation, whereas those with a chronic leukemia are often diagnosed incidentally because of abnormal blood cell counts. White blood cell counts above 100,000 per mm3 (100 x 10(9) per L) represent a medical emergency because of the risk of brain infarction and hemorrhage.     

血小板明显波动的难治性贫血伴环状铁粒幼细胞增多的临床与生物学特征

为了探讨血小板明显波动的RARS与RARS—T在临床表现、分子生物学特征及预后转归的相关意义，采用骨髓细胞涂片和骨髓活检观察细胞形态学改变，用流式细胞术检测细胞免疫学特征，染色体分析检测细胞遗传学改变，应用AS—PCR、基因测序检测JAK2V617F、MPLW515L点突变。结果表明：本例患者确诊为RARS，多次发生血栓相关并发症，血钾水平与血小板计数呈正相关。血小板计数增高时，外周血及骨髓涂片中发现巨大畸形血小板，血小板大簇易见；骨髓活检示巨核细胞数量显著增多；JAK2V617F、MPLW515L基因突变均阴性。结论：RARS可向RARS—T转化，伴骨髓巨核细胞增殖、巨大畸形血小板、JAK2 V617F可能为阴性。病程中出现巨大血小板、血小板计数明显波动时同样要高度重视相关血栓事件的防治，监测相关基因突变。     

儿童不典型再生障碍性贫血与免疫性血小板减少性紫癜的特点及早期鉴别诊断

目的分析并比较不典型再生障碍性贫血(aplastic anemia,AA)和特发性血小板减少性紫癜(idiopathic chrombocytopenic purpura,ITP)的外周血及骨髓特点,为临床诊断提供一定依据,减少误诊的发生。方法选取2008年1月～2014年12月西安交通大学医学院第二附属医院收治的不典型再生障碍性贫血15例为不典型AA组;同期初诊的急性ITP 30例为ITP组。入院后均行血常规、骨髓涂片,自身抗体系列,抗血小板抗体等检查。所有病例入组前排除白血病,MDS,典型AA等血液疾病。结果不典型AA组患儿中性粒细胞计数为2.17±1.07(ANC),血红蛋白为93.23±32.11 g/L(HB)显著低于ITP患儿(ANC为2.73±0.39 g/L及HB为111.11±42.23 g/L),两组间进行t检验,差异具有统计学意义(P<0.05);而ITP患儿血小板计数为(30.38±2.22)×109/L,显著低于不典型再障患儿(47.14±2.17)×109/L,两组间进行t检验,差异具有统计学意义(P<0.05);不典型AA患儿巨核细胞数为17.83±7.83明显低于ITP患儿225.3±64.23,两组间进行t检验,差异具有统计学意义(P<0.05)。结论不典型AA和ITP存在许多相似性,容易发生误诊,当患儿出现血小板降低时,应仔细分析其他血细胞的特点,并参照骨髓穿刺结果,必要时进行骨髓活检以明确诊断。     

成人脓毒症患者骨髓细胞学特征的研究

目的探讨脓毒症患者骨髓细胞学特征及其临床意义。 方法回顾性分析2015年1月至2020年10月在中山大学孙逸仙纪念医院重症医学科和全科医学科住院,诊断为脓毒症并行骨髓细胞学检查的57例患者的骨髓象特征,分析其与外周血血象、感染指标、临床转归和预后的相关性。 结果脓毒症患者外周血血象表现多样,白细胞计数从极度降低到明显升高不等,多表现为中度贫血、血小板减少、淋巴细胞减少;骨髓象以有核细胞增生活跃或明显活跃为主,占82.5%（47/57）,少数患者增生减低;粒红比例升高,多表现为中性杆状粒比例下降,中性分叶粒比例升高,伴部分中性粒胞浆颗粒增粗、增多或可见空泡,部分可见过分叶现象;部分伴有网状吞噬细胞增多,可见噬血现象;多数外周血涂片中性粒细胞碱性磷酸酶（NAP）染色阳性率和积分升高。脓毒症患者骨髓粒系增生情况与外周血粒细胞计数、骨髓巨核细胞计数与外周血血小板计数均存在正相关（r = 0.324、0.574,P均< 0.05）;大肠杆菌感染与脓毒症患者的骨髓增生情况密切相关（χ² = 13.434,P < 0.001）。 结论脓毒症患者多为感染较重阶段,感染发展至脓毒症,骨髓功能在一定程度上呈耗竭状态,部分增生明显低下,其与外周血象、感染具有一定相关性。脓毒症患者血小板减少与骨髓巨核细胞功能直接相关,脓毒症骨髓巨核细胞减少或成熟障碍为脓毒症血小板减少症发病机制之一。此外,大肠杆菌感染的脓毒症患者多表现为骨髓增生减低。     

Splenic thrombopoiesis after bone marrow ablation with radiostrontium: a murine model

Murine platelet production is normally supported by high-ploidy bone marrow megakaryocytes without significant contribution from splenic megakaryocytes with predominantly low-ploidy levels. We produced sustained bone marrow ablation using radiostrontium, and examined the processes by which splenic platelet production is initiated and maintained in the absence of bone marrow function. Bone marrow hematopoiesis, measured by total nucleated cell number and viability, megakaryocyte colony-forming cells, and granulocyte-macrophage colony-forming cells, was rapidly ablated in mice by using yttrium 90-free strontium 90. Platelet count declined from normal (1224 x 10(3)/microliters) to a nadir (98 x 10(3)/microliters) 11 days after 90Sr, and then rose to a stable level (705 x 10(3)/microliters) on days 20 through 115. Peripheral leukocyte concentration decreased rapidly and remained below 25% of normal in contrast to hemoglobin levels, which were minimally lowered. Mean spleen weight rose rapidly after 90Sr to 66% above normal. Splenic megakaryocyte frequency, measured by two-color fluorescence-activated flow cytometry, rose from basal levels (0.09% +/- 0.06%) to 0.15% +/- 0.07% (p less than 0.001), total spleen nucleated cells fell to 71% of normal, and the absolute number of spleen megakaryocytes was unchanged. Total spleen megakaryocyte colony-forming cells were not significantly increased above normal whereas total spleen granulocyte-macrophage colony-forming cells increased abruptly after day 13 to 10 times normal levels. Splenectomy after hematopoietic recovery from 90Sr bone marrow ablation resulted in a rapid decline of platelet levels, followed by death. Although the spleen became the sole site of platelet production, the splenic megakaryocyte ploidy distribution was only minimally changed from normal, and the modal ploidy class remained 2N. In contrast to experimental thrombocytopenia in mice with intact bone marrow, in which megakaryocyte ploidy is increased, thrombocytopenia associated with sustained bone marrow ablation does not result in upward regulation of splenic megakaryocyte ploidy as a compensatory mechanism.     

50例存活三年以上重型再生障碍性贫血患者的长期随访

目的 评价重型再生障碍性贫血（SAA）患者免疫抑制治疗（IST）后的远期疗效。方法 对50例IST后存活3年以上SAA患者长期随访，了解其造血重建（血常规、骨髓象、骨髓活检、造血祖细胞体外培养）、T淋巴细胞免疫、生活质量及治疗不良反应等情况。结果 IST后各随访时间（3年、4年、5年以上）的患者中分别有81．3％（13例）、86．7％（13例）、89．5％（17例）血常规正常，93．4％（15例）、93．3％（14例）、94．7％（18例）骨髓象正常，37．5％（6例）、40．0％（6例）、73．7％（14例）造血祖细胞培养正常；全部患者中有86．0％（43例）血常规正常、94．0％（47例）骨髓象正常、52．0％（26例）造血祖细胞培养正常，88．0％（44例）Karnofsky评分达100分。各随访时间共有31例患者进行了骨髓活检，其中26例（83．9％）正常；37例患者进行了T淋巴细胞亚群检查，其中29例（78．4％）正常。未发现晚期克隆性疾病。治疗近期不良反应轻微。24例患者的全部随访检查结果正常。结论 部分SAA患者经IST后，造血功能、T淋巴细胞免疫、生活质量可完全恢复，且无显著不良反应，即达治愈。     

Clinical approaches involving thrombopoietin to shorten the period of thrombocytopenia after high-dose chemotherapy

High-dose chemotherapy followed by a peripheral blood stem cell transplant is successfully used for a wide variety of malignancies. A major drawback, however, is the delay in platelet recovery. Several clinical strategies using thrombopoietin (Tpo) have been developed in an attempt to speed up platelet repopulation. In contrast to its success in immune thrombocytopenia and in low-dose toxic chemotherapeutic regimens, Tpo appears less effective in the case of high-dose chemotherapy and peripheral blood stem cell transplant. To develop a successful therapeutic approach, more knowledge is needed on several aspects of megakaryocyte (progenitor) biology, such as homing to the bone marrow, endomitosis, and platelet formation. Interactions of the megakaryocytes with the marrow vasculature and the microvascular microenvironment are other key factors for optimal thrombocytopoiesis. The present report reviews the background of the inefficiency of Tpo after intensive chemotherapy and describes possible strategies that might lead to successful therapies to treat chemotherapy-induced thrombocytopenia.     

儿童感染相关性噬血细胞综合征37例临床分析

目的分析儿童感染相关性噬血细胞综合征的临床特点及诊治情况。方法回顾性分析37例感染相关性噬血细胞综合征病儿的临床及实验室资料。结果EB病毒感染者23例（62．2％）,CMV感染者4例（10．8％）,细菌感染者8例（21．6％）,病原不明者2例（5．4％）;本组病例临床主要特点为持续高热（100．0％）,肝大（83．3％）,脾大（91．7％）;外周血白细胞计数减少（76．2％）,血小板减少（91．7％）,血红蛋白减少（77．8％）;纤维蛋白原低下（66．7％）;肝功能异常（51．2％）,血清三酰甘油升高（100．0％）,铁蛋白升高（83．3％）;骨髓涂片找到噬血细胞（100．0％）。VP-16、环孢素A（CsA）、糖皮质激素及静脉注射免疫球蛋白（IVIG）联合治疗33例,糖皮质激素及IVIG联合治疗2例,其中治疗有效30例,死亡5例。确诊后放弃治疗2例。结论典型的儿童感染相关性噬血细胞综合征临床以持续高热、肝脾大为主要表现,实验室检查以白细胞计数、血红蛋白及血小板中两系或三系减少、低纤维蛋白血症、高三酰甘油血症、肝功能异常及血清铁蛋白升高,骨髓找到噬血细胞为主要特点。早期诊断、早期治疗能挽救该类病儿的生命。     

Pharmacologic eigenvalues: beating the rap on bone marrow failure

Patients suffering from sustained acute or chronic illness often have decreased white blood cell and platelet counts as well as anemia, and bone marrow studies routinely show only decreased numbers of blood precursor cells. While much has been recently learned about the cause of isolated anemia, the pathogenesis of true bone marrow failure (i.e., low bone marrow cellularity and low counts in multiple blood lineages) has remained elusive. In this issue of the JCI, Chen et al. present evidence that overactivation of mammalian target of rapamycin signaling in HSCs is found in two mouse models of bone marrow failure, and they show that treatment with rapamycin significantly normalizes the low blood counts.