小儿急性白血病患者血浆纤维结合蛋白测定的临床意义

小儿急性白血病患者血浆纤维结合蛋白测定的临床意义张宝玺，郭稳捷河北医学院附属二院儿科ＴＨＥＣＬＩＮＩＣＡＬＳＩＧＮＩＦＩＣＡＮＣＥＯＦＰＬＡＳＭＡＦＩＢＲＯＮＥＣＴＩＯＮＤＥＴＥＣＴＩＯＮＩＮＣＨＩＤＨＯＯＤＡＣＵＴＥＬＥＵＫＥＭＩＡ￥ＺｈａｎｇＢａ...     

小儿白血病形态学、免疫学分型研究

小儿白血病形态学、免疫学分型研究程岩，田根全，袁文菊，杨小红青海省儿童医院ＴＨＥＲＥＳＥＡＲＣＨＯＦＭＯＲＰＨＯＬＯＧＩＣＡＮＤＩＭＭＵＮＯＬＯＧＩＣＡＬＣＬＡＳＳＩＦＩＣＡＴＩＯＮＯＦＴＨＥＰＥＤＩＡＴＲＩＣＬＥＵＫＥＭＩＡ￥ＹａｎＣｈｅｎｇｅｔａ...     

小儿恶性组织细胞增生症血象及骨髓象分析

小儿恶性组织细胞增生症血象及骨髓象分析郭艺杰，王令仪武汉同济医科大学同济医院儿科ＡＮＡＬＹＳＩＳＯＦＨＥＭＯＧＲＡＭＡＮＤＢＯＮＥＭＡＲＲＯＷＰＩＣＴＵＲＥＳＩＮＭＡＬＩＧＮＡＮＴＨＩＳＴＯＣＹＴＯＳＩＳ￥ＧｕｏＹｉＪｉｅ，ｅｔａｌ（Ｄｅｐａｒｔｍｅ...     

血管内溶血性贫血多指标联合诊断优化方案的探讨

血管内溶血性贫血多指标联合诊断优化方案的探讨卢桂森，张华，陈琼俊广西玉林地区红十字会医院ＣＯＭＢＩＮＥＤＤＩＡＧＮＯＳＴＩＣＯＰＴＩＭＩＺＩＮＧＳＨＥＭＥＷＩＴＨＭＵＬＴＩＰＬＥＩＮＤＥＸＥＳＦＯＲＩＮＴＲＡＶＡＳＣＵＬＡＲＨＥＭＯＬＹＴＩＣＡＮＥＭ...     

小儿特发性血小板减少性紫瘢T细胞亚群的观察

小儿特发性血小板减少性紫瘢Ｔ细胞亚群的观察田根全，袁文菊，程岩青海省儿童医院ＴＨＥＴ－ＣＥＬＬＳＵＢ－ＧＲＯＵＰＭＥＡＳＵＲＥＭＥＮＴＯＦＩＤＩＯＰＡＴＨＩＣＴＨＲＯＭＢＯＣＹＴＯＰＥＮＩＣＰＵＲＰＵＲＡ（ＩＴＰ）ＩＮＰＥＤＩＡＴＲＩＣＰＡＴＩＥＮＴ...     

Y-1型计测板对红细胞微量压积测量效果的初步评价

Ｙ－１型计测板对红细胞微量压积测量效果的初步评价袁光孚，保菊英，王冀湘，耿广献西宁市一医院ＥＶＡＬＵＡＴＩＯＮＯＦＡＣＡＬＣＵＬＡＴＩＮＧＰＬＡＴＥＭＯＤＥＬＹ－ＩＦＯＲＴＨＥＭＥＡＳＵＲＥＭＥＮＴＯＦＭＩＣＲＯ－ＨＥＭＡＴＯＣＲＩＴ．￥ＹｕａｎＧｕ...     

重组人集落刺激因子在白血病中的临床应用

重组人集落刺激因子在白血病中的临床应用顾立达，艾孜买提，孔佩艳，严媚，赵勒新疆医学院一附院儿科血液组ＣＬＩＮＩＣＡＬＡＰＰＬＩＣＡＴＩＯＮＯＦＲＥＣＯＭＢＩＮＡＮＴＨＵＭＡＮＧＲＡＮＮＬＯＣＹＴＥＣＯＬＯＮＹＳＴＩＭＵＬＡＴＩＮＧＦＡＣＴＯＲＯＮＬＥ...     

血小板质控初探

血小板质控初探袁光孚，张玉芳，保菊英，秦振庭西宁市第一人民医院ＣＡＭＰＡＲＩＳＩＯＮＯＦＤＩＦＦＥＲＥＮＴＭＥＴＨＯＤＳＦＯＲＰＬＡＴＥＬＥＴＣＯＵＮＴＩＮＧＡＴＨＩＧＨＡＬＴＩＴＵＤＥＱＩＮＧＨＡＩ￥Ｋｕａｎｇ－ＦｕＹｕａｎ．ｅｔａｌ（Ｘｉ－Ｎｉｎ...     

不同日龄、不同病情新生儿血小板数量的观察

不同日龄、不同病情新生儿血小板数量的观察秦振庭，王广方北京医科大学第一临床医学院妇儿医院ＰＬＡＴＥＬＥＴＣＯＵＮＴＳＡＴＤＩＦＦＥＲＥＮＴＡＧＥＧＲＯＵＰＳＯＦＮＥＷＢＯＲＮＳ￥Ｃｈｅｎ－ＴｉｎｇＣｈｉｎｅｔａｌ（Ｗｏｍｅｎ＆Ｃｈｉｌｄｒｅｎｈｏｓｐ...     

新生儿依赖维生素K凝血因子活性的动态观察

新生儿依赖维生素Ｋ凝血因子活性的动态观察楼金吐，俞锡林，孔宏伟浙江医科大学附属儿童医院ＴＨＥＣＨＡＮＧＩＮＧＰＡＴＴＥＲＮＯＦＶＩＴＡＭＩＮＫ－ＤＥＰＥＮＤＥＮＴＦＡＣＴＯＲＳＩＮＮＥＯＮＡＴＥＳ￥ＬｏｕＪｉｎｔｕ，ｅｔａｌ．（Ｉｎｓｔｉｔｕｔｅｏｆ...     

骨髓活检在小儿再生障碍性贫血临床应用的研究

为探讨骨髓活检在小儿再生障碍性贫血（简称再障）临床应用的价值，采用骨髓活检改良制片法对88例再障患儿进行骨髓组织学定量分析并与涂片对照；同时对其中30例进行治疗后观察；并对76例进行随访。结果表明：小儿再障诊断符合率和疗效判定骨髓活检高于涂片检查，疗效与骨髓造血组织容量，造血细胞绝对值呈正相关，而与非造血细胞绝对值呈负相关；初检造血组织容量大小与生存期长短有关。结果提示：骨髓活检改良制片法适用于儿童，其可作为小儿再障早期诊断、疗效判定、预后评估的一个客观指标。     

小儿急性淋巴细胞性白血病伴继发骨髓纤维化及疗效观察

对1991年10月至1996年2月收治的74例小儿急性淋巴细胞性白血病（ALL）同时作骨髓（BM）涂片与切片（活检）检查，发现11例伴有继发骨髓纤维化（SMF）。其中5例BM涂片与切片增生度不相符，涂片增生度减低或极度减低，切片示增生活跃至极度活跃（相差2～5个级别）；4例BM涂片未达ALL诊断标准，原淋＋幼淋＜0．3（0．08～0．25），切片见大量幼稚细胞浸润；6例外周血白细胞＜3×109/L（1．3～2．79×109/L）。4例早期用VLP方案为主治疗，持续完全缓解时间为16～30个月，疗效良好。2例早期用VCP方案治疗，一直未能完全缓解。BM切片示脂肪组织增多（70％和75％），疗效差。结果表明：BM切片与涂片结合对小二ALL伴SMF的诊断有重要价值；小儿ALL伴SMF的发生率可能不低；其治疗的成功与否与化疗早期的选择关系密切。     

Visceral leishmaniasis: bone marrow biopsy findings

Visceral leishmaniasis (VL) or Kala-azar is a common parasitic infection among children in Iran. The records of 249 children with VL were evaluated retrospectively. The clinical, hematologic, and bone marrow biopsy findings were studied. In particular, we assessed whether there was an association between bone marrow biopsy findings and prognosis. Five major groups were identified: (1) hypercellular marrow with many Leishman Donovan (LD) bodies, (2) multiple noncaseating granulomas with a few LD bodies, (3) diffuse fibrosis with rare LD bodies, (4) benign lymphoid nodules with many LD bodies, and (5) marrow necrosis with many LD bodies. The patients with hypercellular marrow and benign lymphoid nodules were alive and responded well to glucantime therapy. The patients with marrow fibrosis and marrow necrosis died and were resistant to any type of therapy. Patients with granulomas did not respond to glucantime therapy but responded to amphotericin B. However, less than half of the patients died owing to malnutrition and misdiagnosis. We correlated the bone marrow biopsy findings with the treatment outcomes and prognosis. The outcome was excellent in cases of hypercellular marrow, very poor in cases of fibrosis and necrosis, and intermediate in cases of granulomas. As a result, we believe that bone marrow biopsy findings can be helpful for assessing the prognosis of VL patients.     

再生障碍性贫血患儿骨髓祖细胞培养的临床意义

目的探讨再生障碍性贫血(AA)患儿红细胞集落生成单位(CFU-E)、爆式红细胞集落生成单位(BFU-E)、粒细胞-单核细胞集落生成单位(CFU-GM)及巨核细胞集落生成单位(CFU-Meg)骨髓祖细胞培养情况,了解骨髓祖细胞在AA发病中的作用及意义。方法抽取28例AA患儿骨髓3～4mL,以淋巴细胞分离液提取单个核细胞,用磷酸盐缓冲液(PBS)洗涤2次,调节单个核细胞水平为106/mL,取0.3mL分别接种于红系、粒-单核系、巨核系培养基分别作4类祖细胞培养,在7、14d测定其集落数。结果28例AA中,4类集落数均值与对照组比较,差异有显著意义(P<0.01或P<0.001)。集落数随病情加重而逐步下降。4类集落数低于对照组患儿下限所占百分比分别为BFU-E35.71%、CFU-E85.71%、CFU-GM75.00%、CFU-Meg89.29%。治愈及进步者4类集落数接近对照组水平,治疗无效及进展者与治疗前比较无变化。结论动态监测AA患儿骨髓4类祖细胞集落数变化对辅助诊断、观察疗效及预后判断均有指导意义。     

儿童再生障碍性贫血-阵发性睡眠性血红蛋白尿综合征临床分析

为探讨儿童再生障碍性贫血 -阵发性睡眠性血红蛋白尿 (AA PNH)综合征的临床及实验室检查特点 ,特总结近年我科收治的 5例AA PNH综合征患儿的病历资料 ,从临床、血象、骨髓象、溶血方面的检查、CD5 9+细胞等方面分析其特点。结果显示儿童AA PNH综合征以学龄儿童为多见 ,本文报导 1例发病年龄 1岁半 ,为目前文献报导中年龄最小者 ;儿童AA PNH以AA伴PNH特征多见 ;临床上有严重贫血、肝脾稍大等特点 ;实验室检查可见全血细胞减少、Ret大多不低或升高、血片中可见有核红细胞 ;骨髓红系增生 ,粒红比例倒置、巨核系统大多增生低下 ;Hams试验、蔗糖水溶血试验、尿Rous试验、蛇毒因子 (Cof)溶血试验可阳性 ;血细胞膜CD5 9-细胞增多 ,粒细胞膜CD5 9-细胞比红细胞膜出现早。因此 ,可以结论　儿童AA PNH综合征多见于学龄期 ,以AA伴PNH特征多见。临床上对于全血细胞减少、骨髓红系增生、血Ret升高、血片中可见有核红细胞的患儿 ,应进一步做与PNH有关的检查 ,并综合分析 ,CD5 9-粒细胞增多具有早期诊断价值     

Hemogram and bone marrow morphology in children with chronic aplastic anemia and myelodysplastic syndrome

Background  Aplastic anemia (AA) and myelodysplastic syndrome (MDS) are both acquired disorders in which bone marrow fails to produce or release sufficient blood cells. Anemia, infections and thrombocytopenia are common signs of such diseases. Clinically, it is difficult to distinguish chronic aplastic anemia (CAA) from MDS, especially from MDS without splenomegaly. As prognosis and treatment of AA and MDS are different, it is extremely important to make a differential diagnosis for the two diseases. Methods  The medical records of 31 patients with CAA and 17 patients with MDS were retrospectively reviewed. Hemogram, bone marrow smear and biopsy for those patients were analyzed. Results  The mean counts of monocytes and platelets in the peripheral blood of the CAA patients were significantly lower than those of the MDS patients. Bone marrow smear showed a reduction of cellularity in CAA patients. The mean counts of myeloblasts+promyelocytes, myeloblasts+proerythroblasts, and megakaryocytes in the bone marrow of CAA patients were markedly lower than those in MDS patients. But the mean lymphocyte count was reversed. Bone marrow cells showed morphological abnormalities in MDS. Hematopoietic tissue in the bone marrow biopsy decreased obviously in more than 96% of the patients with CAA. Adipose tissue in the bone marrow of CAA patients increased obviously. A reduction or deficiency (<2 cell/piece) of megakaryocytes was noted in 28 patients with CAA. Fibrous tissue in the bone marrow was detected in 5 patients with CAA. Bone marrow biopsy results showed hypercellular changes in 12 MDS patients. Ten patients showed aggregated erythroblasts which were in the same stage of development, and 15 patients had abnormal localization of immature precursors (ALIP). Conclusions  Blood cell counts can be decreased in addition to the reduction of cellularity in the bone marrow without dyshematopoiesis in CAA patients. Peripheral blood monocytes, fibrous tissue and cellularity in bone marrow are increased in MDS. Dyshematopoiesis and ALIP may appear characteristically in the children with MDS. Histology of bone marrow is important in the differential diagnosis of MDS and CAA.     

Main causes of aplastic anemia misdiagnosed as immune thrombocytopenia in children北大核心CSCD

目的归纳曾被误诊为免疫性血小板减少症(ITP)的50例儿童再生障碍性贫血(再障)的主要原因和鉴别诊断经验,以供临床参考。方法参照儿童再障和ITP诊断标准,回顾性分析2007年1月至2020年12月上海市同济医院儿科收治的外院误诊病例的病初资料和本院复诊检测结果,归纳误诊原因和鉴别诊断要点。结果在同期收治的165例儿童再障中,共有50例(30.3%)曾被误诊为ITP。分析归纳主要误诊原因为:1.临床表现不符合"典型ITP",未按照国际指南标准行必要的骨髓检查以明确诊断,共22/50例。2.骨髓检测结果解读有误。在28例初诊行骨髓涂片检查者中,6例(21%)骨髓显示典型再障骨髓象,但仍被诊断为ITP。3.骨髓涂片结果不典型者,未行骨髓活检以助诊断(15/28例,54%)。4.初诊时符合ITP诊断标准,但经糖皮质激素等治疗无效后,未行必要复查以核实诊断(7/28例,25%)。结论临床应严格参照执行相关疾病诊断标准,以避免经验性错误。诊断ITP需要慎重,尤其是临床表现不典型,或一线药物无效者,必须进行骨髓检查(必要时行骨髓活检),并按诊断标准正确解读检测结果,以避免临床误诊或漏诊。     

An aggressive bone marrow evaluation including immunocytology with GD2 for advanced retinoblastoma

There is general agreement that bone marrow (BM) examination for staging in patients with retinoblastoma should be limited to cases with advanced disease. However, there are limited data about the yield of sampling multiple sites with aspirations and biopsies and immunocytology. Our policy for BM examination included: 2 aspirates and 2 biopsies at the posterior iliac crest scheduled only for cases with postlaminar optic nerve extension (n=56), scleral invasion (n=10) or orbital (n=5) or metastatic disease at diagnosis (n=7) or at extraocular relapse (n=18). Immunocytology with the antibodies 3A7 or 3F8 for the ganglioside GD2 was performed. From 1/1994 to 3/2005, 277 newly diagnosed patients and 5 at extraocular relapse were included. BM invasion was not found in any of the 66 patients enucleated with disease confined to the globe, but was found in 11/27 of those with overt extraocular disease. There were 2/11 cases with at least 1 negative aspirate with positive biopsy and/or immunocytology for GD2. GD2 positivity was found in 9/9 cases. A more aggressive BM evaluation has a low yield in enucleated patients with high-risk features but disease limited to the globe. However, in cases with overt extraocular dissemination, the use of BM biopsy and immunocytology for GD2 allowed for the detection of cases that would have been missed by aspirations alone. GD2 was intensively expressed and it may also be used to monitor disease response and the presence of minimal residual disease.     

腺泡状横纹肌肉瘤骨髓转移后细胞形态特点分析及细胞化学染色鉴别

目的探讨腺泡状横纹肌肉瘤(alreolar rhabdomyosarcoma,ARMS)骨髓转移后细胞形态特点,以及应用细胞化学染色对骨髓中横纹肌肉瘤(rhabdomyosarcoma,RMS)细胞进行鉴别诊断。方法对1例ARMS骨髓转移患者的肿瘤原发部位进行病理检查并相关病理免疫组织化学染色。抽取患者胸骨骨髓细胞涂片,采用常规瑞氏染色、过氧化物酶染色、糖原染色及结蛋白染色,对骨髓中RMS细胞形态特点进行分析及细胞化学染色鉴别。结果病理活检与免疫组织化学染色结果符合ARMS病理特征,骨髓细胞形态及细胞化学染色结果符合RMS细胞特征。结论ARMS骨髓转移后,骨髓细胞涂片中RMS细胞形态特点明显,同时结合骨髓细胞化学染色结果,能够较准确地对骨髓中RMS细胞进行识别及诊断。     

14例小儿骨髓坏死临床特点及实验室诊断分析

目的分析近八年郑州大学第一附属医院儿科骨髓坏死(BMN)病例,了解BMN在儿科的发病特点。方法对1996年1月至2003年9月14例小儿BMN的临床表现,实验室检查及原发病诊断进行比较,并做回顾性分析总结。结果12例(86%)并发于恶性疾病,其中6例原发病为急性淋巴细胞性白血病(ALL),神经母细胞瘤(NB)和急性髓细胞性白血病(AML)各2例,非何杰金淋巴瘤(NHL)和MDS各1例;2例(14%)并发于非恶性疾病,其中一例为结核感染,另一例为金黄色葡萄球菌败血症。最显著的临床表现依次为:骨痛12例,发热11例,苍白10例,黄疸7例,皮肤粘膜出血6例。最常见的实验室发现依次为贫血10例,血小板减少7例;骨髓细胞学分析发现轻度骨髓坏死2例,中度4例,重度8例。结论恶性血液肿瘤和恶性转移瘤是小儿BMN的最常见病因,另外严重感染也是小儿BMN的常见病因之一。