小儿急性造血功能停滞14例

急性造血功能停滞（又名急性红细胞再生障碍性贫血、急性再障危象等），小儿少见。本文报道本单位十年来收治的１４例。男１２例女２例，最小年龄６月、最大１２岁，均有一定致病因素，以感染、药物毒性作用稍多。以中、重度贫血为多，网织红细胞明显减低。骨髓象：有核细胞增生活跃１３例，增生减低１例；但粒／红之比最低为１９：１，最高为１０１：１，多数介于３０￣５６：１之间。重点讨论了病因、早诊线索及确诊。造血功能停滞     

急性造血功能停滞的研究进展

急性造血功能停滞是由于多种原因所致的骨髓造血功能停滞。临床表现以贫血为主，血中红细胞及网织红细胞减少或三种血细胞均减少。此病可于短期内自然恢复。     

小儿再生障碍性贫血伴骨髓细胞病态造血现象临床研究

再生障碍性贫血(AA)是血液系统中较少见的,以造血细胞干细胞损伤,外周全血细胞减少为特征的疾病,一般骨髓中不会出现病态造血现象,但在我们最近的观察中发现,有些AA病人也会出现。AA和骨髓增生异常综合征(MDS)在临床表现上尤为相似,给医生诊断及治疗带来阻碍。那么这种有病态造     

造血干细胞移植治疗再生障碍性贫血

再生障碍性贫血(aplastic anemia,AA)是一种儿童常见的骨髓造血功能衰竭症.临床主要表现为骨髓造血功能低下、全血细胞减少和贫血、出血、感染以及相关症候群.     

感染后急性造血功能停滞1例

患儿,女,6岁,住院前10日曾患急性肠炎、急性支气管肺炎、急性咽喉炎、右侧下眼睑脓肿等多种感染。在当地卫生所治疗4日,因面色苍白,气短、乏力、食欲减退,高热等转我院。急查末稍血Hb45g/L,WBC1.1×10~9/L,分类中性0.04,L0.96,RBC1.50×10~(12)/L,Ret0.001。查体:     

脐血造血干细胞移植治疗儿童血液病14例临床观察

脐血造血干细胞移植可以根治儿童白血病、再生障碍性贫血、血红蛋白病及先天性免疫缺陷等疾病。自1988年Gluckman等首先应用脐血移植（CBT）成功治疗1例儿童Fanconi贫血以来,CBT发展迅速。由于脐血来源广、采集方便、对供者无害、移植物抗宿主病（GVHD）发生率低而得到广泛应用。我们采用脐血造血干细胞移植治疗14例儿童血液病,现报告如下。     

急性造血功能停滞的研究进展

急性造血功能停滞是由于多种原因所致的骨髓造血功能停滞 ,临床表现以贫血为主 ,血中红细胞及网织红细胞减少或三种血细胞均减少。此病可于短期内自然恢复。194 2年Lunger发现遗传性球形红细胞增多症患儿的贫血危象常由于红细胞生成减少 ,而并非都是由于溶血增多。 194 8年Owren把这种病命名为再生障碍危象 (aplasticcrisis) ,并且描述了它的自然过程 :一般先有轻度感染 ,以后骨髓红系再生障碍 ,短期内恢复 ,并有反跳的过度增生。194 9年Gasser描述一种相似的疾病 ,发生于非溶血的患者 ,此后国内外文献报告了很多病例[1] 。在我国多将这种…     

IDIOPATHIC APLASTIC ANAEMIA IN CHILDREN

The results of treatment of 19 children with idiopathic aplastic anaemia is reported. Initially the patients received testosterone-prednisone therapy. When a satisfactory haemoglobin level had been reached metandienon was substituted for testosterone and the corticosteroids withheld. 8 patients died without signs of response, 2 patients responded initially but died later. The remaining 9 patients are alive but in only 3 of them was it possible to discontinue all therapy without signs of relapse. The rate of remission varied considerably. One patient required 23 months of combined therapy to achieve a normal haemoglobin level. A moderate hypoplasia of the bone^marrow at the start of therapy was the most favourable prognostic sign in this series. Therefore, it may be concluded that every effort should be made to diagnose the disease before the bone-marrow changes have progressed to severe hypoplasia.     

遗传性球形红细胞增多症伴家族性一过性造血障碍危象

在同一家族内和相邻社区里，在同一时间里发现遗传性球形红细胞增多症并发一过性造血障碍儿童3例，对早期和恢复期的周围血和骨髓造血作了连续观察，表明早期有红系造血抑制，越是接近成熟的幼红细胞抑制越多，并可见巨形嗜碱性原始红细胞，恢复期中，有中、晚幼红细胞高度增生。     

ACUTE POISONINGS OF CHILDREN IN OSLO

ABSTRACT. A one year prospective study of all children under 15 years of age presenting for acute poisoning in Oslo is reported. There were 181 admissions in 179 children, of which 97 (54 %) were boys, giving an annual incidence of 2.3 %. All children survived without sequelae. Most poisonings were accidental and only two suicidal attempts were recorded. Of all poisonings 68 per cent occurred between the age of 1 and 2 years. The dominating toxic agents were drugs (44 %), tobacco (22 %) and petroleum products (9 %). Most poisonings were mild and only 7 (4 %) classified as severe. Seventy-two per cent of all children were admitted within the second hour after the ingestion of the toxic agent. Therapy should therefore be directed towards emptying the stomach with emetics or gastric lavage, unless corrosives or petroleum products are ingested. Childhood poisonings still call for better preventive measures since the toxic agent was found inappropriately stored in 86 % of the accidental poisonings.     

L-ASPARAGINASE TREATMENT OF ACUTE LEUKAEMIA IN CHILDREN

The results of asparaginase-prednisone therapy in children are reported. In 12 children with untreated acute lymphoblastic leukaemia 9 remissions were obtained, there were 2 failures and in one patient therapy had to be stopped because of an anaphylactic shock. One child with an acute myelocytic-granulocytic type of acute leukaemia did not respond. One child with an acute monocytic-monoblastic leukaemia was given asparaginase only. He responded very well. Four patients with a relapse of an acute myeloblastic leukaemia were treated: there were two failures, one child died too early from an infection to be evaluated. One child showed a very good reaction. Serious side-effects were few, the drugs gave many biochemical disturbances including a constant hypofibrino-genaemia, but these were well tolerated and reversible.     

THE BENEFIT OF ATG IN IMMUNOSUPPRESSIVE THERAPY OF CHILDREN WITH MODERATE APLASTIC ANEMIA

Background. Previous studies specifically focused on the immunosuppressive therapy (IST) of children with moderate aplastic anemia (MAA) are rare. The aim of this study was to evaluate the advantage of using antithymocyte globulin (ATG) in the IST and its outcome of children with MAA. Methods. Forty-two children diagnosed with moderate aplastic anemia from 1993 to 2006 were retrospectively reviewed. Eighteen patients treated with ATG, cyclosporin A (CSA), and androgen are defined as the ATG group, the other 24 patients treated with CSA and androgen are defined as the non-ATG group. Survival and hematological response of the two groups were studied. Results. Response rate and transfusion-independent survival of the ATG group were both significantly higher than those of the non-ATG group (83.33 vs. 41.7%, p =. 006; and 83.33 vs. 50%, p =. 043, respectively). Compared with non-ATG group, fewer patients in ATG group progress to severe aplastic anemia (p =. 03). Conclusion. Immunosuppressive therapy including ATG benefits children with moderate aplastic anemia.     

48例儿童骨髓增生异常综合征的细胞学诊断

观察分析儿童MDS外周血象及涂片和骨髓细胞病态改变 ,用血细胞分析仪进行外周血细胞计数和瑞氏染色法进行血及骨髓细胞形态学分析。结果显示 :血细胞计数大多为血红蛋白、白细胞和血小板减少。血涂片染色 ,部分可见原始和幼稚白细胞和 (或 )幼红细胞。骨髓粒、红、巨红三系均有不同程度的病态造血表现 :粒系原始细胞增多 ,比值及形态异常 ;半数以上患者红系过多 ,并有明显形态异常 ;巨核细胞计数减少 ,小巨核检出率为 0 .1 8。我认为 ,儿童MDS至少有两系以上病态改变 ,与成人MDS虽然诊断标准相同 ,但儿童MDS有其发病及诊断特点。     

大剂量丙种球蛋白静脉输注治疗儿童再生障碍性贫血的观察

应用大剂量丙种球蛋白（lg／kg·次，3～4周1次，共6次）治疗儿童再生障碍性贫血13例，有效率76．9％，有效病例CD4／CD3由治疗前倒置恢复到正常状态。对照组有效率54．5％，CD4／CD8有所增高，但未能恢复到正常。说明大剂量丙种球蛋白能纠正再障的T淋巴细胞紊乱状态，是治疗儿童再障的有效方法。     

NEW PERSPECTIVES ON THE DIFFERENTIATION OF APLASTIC ANEMIA AND MDS IN CHILDREN

Due to recent advances in immunosuppressive therapy, the prognosis for aplastic anemia (AA) has improved dramatically. As more patients with this disease have been surviving longer, the number of AA patients who develop myelodysplastic syndrome (MDS) or leukemia has increased. However, it is unclear whether the development of this type of late clonal disorder is related to a particular therapy, and whether hypoplastic MDS can be differentiated from AA at diagnosis. Although there are no definitive answers to these questions, some forms of AA and MDS are likely within the same spectrum of disease. Thus, when treating AA, it is essential to evaluate various clinical and laboratory information, including cell morphology and chromosomal aberration. This paper discusses acute myelocytic leukemia (AML) and MDS in children diagnosed as having AA, and it presents problems associated with the diagnosis of this disease.