儿童急性白血病完全缓解后骨髓幼稚细胞低比例增高的临床意义

目的　了解急性白血病患儿完全缓解 (CR)后骨髓幼稚细胞比例出现 0 0 5～ 0 2 5时对预后的影响 ,为临床治疗策略的调整提供依据。方法　对 1998～ 2 0 0 1年上海儿童医学中心收治急性白血病患儿CR后按骨髓幼稚细胞比例分为A组 (<0 0 5 )和B组 (0 0 5～ 0 2 5 ) ;B组又分为B1(0 0 5～ 0 10 )、B2 (～ 0 15 )、B3(～ 0 2 5 ) 3个亚组 ,分析各组与急性白血病复发的关系。结果　急性淋巴细胞白血病 (ALL)患儿CR后骨髓幼稚细胞≥ 0 10时 ,复发率与阴性对照组差异有显著性 ;急性非淋巴细胞白血病 (ANLL)患儿CR后骨髓幼稚细胞≥ 0 15时 ,复发率与阴性对照组差异有显著性。结论　ALL患儿CR后骨髓中原始淋巴细胞 幼淋巴细胞≥ 0 10及ANLL患儿CR后骨髓中原始粒细胞 早幼粒细胞或原始单核细胞 幼单核细胞≥ 0 15时应考虑及时调整治疗方案。     

Fungal pneumonia: the predominant lung infection causing death in children undergoing bone marrow transplantation

The study included 6 children (aged 4–14 years) receiving a conditioning regimen for bone marrow transplantation (BMT) and 14 children (aged 2-14 years) with bone marrow transplants (13 allo-geneic, 1 autologous). The children underwent flexible fibre-optic bronchoscopy (FFB) with broncho-alveolar lavage during 6 and 17 episodes of pneumonia, respectively. The aim was to compare the results of the two groups with respect to bronchoscopy findings, pneumonia-causing agents and outcome. During the conditioning regimen, the aetiological agents were recovered by bronchoscopy in 1/6 (17%) episodes and revealed by autopsy in another episode. In three episodes where the aetiology was uncertain, bacterial pneumonia was suspected in two, and Candida pneumonia in one. In episodes after transplantation the aetiological agents were recovered from bronchoscopy material in 14/17 (82%) patients. Autopsy confirmed the premortal diagnosis in the four children who died. In three episodes, bacterial pneumonia was clinically suspected. Based on clinical manifestations, FFB and autopsy findings, bacterial and fungal pneumonia were the most common diagnoses both during conditioning and after BMT. Fungal pneumonia was the most common cause of death in both groups.     

MRI diagnosis of bone marrow relapse in children with ALL

Background Diffuse marrow replacement in acute leukemia is well known, but there are few reports describing the MRI features of pediatric leukemic relapse. Objective Our purpose was to describe the MRI appearance of pediatric leukemic relapse. Materials and methods A total of 53 consecutive children with a history of ALL were referred for musculoskeletal MRI from 1 January 1998 to 28 February 2007 at one center, and from 1 January 2000 to 2 May 2007 at a second center. From this group, 14 children seen at initial diagnosis of leukemia and 2 children who underwent MRI after therapy for relapse were excluded. The remaining 37 children, 8 with relapse and 29 in remission, were studied. Images of patients with relapse and in remission were reviewed for type and configuration of marrow infiltration; coexisting marrow alterations including osteonecrosis or stress reaction were also reviewed. Results All eight children with relapse demonstrated nodular lesions with well-defined margins. Coexisting osteonecrosis was present in three children (38%) and pathologic fracture in one. Among the 29 children in remission, 9 showed stress reaction/fracture, 14 showed osteonecrosis and 9 showed ill-defined nodules, and in 5 the marrow was completely normal. Conclusion Well-defined nodules in all patients with leukemic relapse suggest that this appearance is characteristic and distinct from the published findings of diffuse marrow replacement in acute leukemia.     

Correlation of lung abnormalities on high-resolution CT with clinical graft-versus-host disease after allogeneic versus autologous bone marrow transplantation in children

Background  Late-onset noninfectious pulmonary complications (LONIPCs) are life-threatening complications of bone marrow transplantation (BMT). Several pathological patterns are described in the literature with different prognoses, and with different relationships to graft-versus-host disease (GVHD). The role of high-resolution CT (HRCT) is not yet well established. Objective  To illustrate different patterns of LONIPCs on HRCT in allogeneic versus autologous BMT in order to investigate the correlation with chronic GVHD (cGVHD). Materials and methods  A total of 67 HRCT scans were performed in 24 patients with noninfectious pulmonary disease at least 3 months after BMT (16 allogeneic, 8 autologous). Abnormality patterns and extension on HRCT images were correlated with the clinical outcome and with the severity of cGVHD. Results  Of 24 patients, 9 showed LONIPCs (1 autologous, 8 allogeneic). There was a significant association between abnormalities on HRCT and severe cGVHD (P = 0.038), with no specific pattern. Prognosis seemed to be related to the severity of cGVHD and not to the extent of abnormalities on HRCT. Conclusion  The significant association between abnormalities on HRCT and severe GVHD suggests that LONIPCs can be a pulmonary manifestation of the disease. HRCT is a useful tool when combined with clinical data.     

Ureteral obstruction following allogeneic bone marrow transplantation in children

Two patients with Ph + CML underwent URD-BMT after conditioning with Bu-Cy-LPAM. They developed hemorrhagic cystitis with an extremely complicated and painful course, caused by ureteral obstruction, requiring prolonged hospitalization. No virus other than cytomegalovirus was found and in both cases was attributed to Cy use. Treatment is usually conservative, but in the case of severe obstruction, a surgical approach should be considered and performed as early as possible to preserve renal function.     

Disease of the liver following bone marrow transplantation in children: incidence, clinical course and outcome in a long-term perspective

Sixty-four consecutive cases of allogeneic ( n = 16), autologous ( n = 47) or syngeneic ( n = 1) bone marrow transplantation (BMT) in children with haematological or lymphoid malignancy, aplasia or metabolic disease were reviewed to assess the incidence, clinical presentation and outcome of liver disease. Median follow-up time was 5 y (1.0-10). No liver diagnosis was established at the pre-transplant check-up. During the first 100d post-transplant, 81% of the patients had impaired liver function as documented by various biochemical parameters. Three of 64 patients (5%) met diagnostic criteria for veno-occlusive disease. Four (25%) of the 16 receiving allografts were diagnosed as having acute graft vs host disease (GVHD) with liver involvement (grades II-III). No patient died of liver disease. During the late post-transplant follow-up, one patient developed HCV hepatitis after packed erythrocyte transfusion. Four patients were diagnosed as having chronic GVHD with liver involvement; three of them also had an episode of CMV hepatitis. At their latest follow-up, the patients with chronic GVHD had aminotransferase values 1.5–3 times the normal, whereas all other long-term survivors had normal or near-normal liver function tests. We conclude that the incidence of serious liver disease was low in this paediatric population of bone marrow recipients.     

Detection of bone marrow involvement by neuroblastoma: Comparison of two cytological methods

In order to improve the assessment of bone marrow (BM) involvement by neuroblastoma, a study has been designated on aspirated material from ten BM sites. The classical method of smearing each BM aspirate (TK1) was compared to cytocentrifugation of the pool of BM samples after gradient density separation (TK2). Twenty smears from each technique were screened for the presence of tumor clumps. The screening was much easier and more rapid by TK2 than by TK1. Of the 103 procedures performed, 100 results were found concordant by both techniques, 3 were found negative by TK1 and positive by TK2. None was found positive by TK1 and negative by TK2. Of the 25 positive procedures, two had an equal number of positive smears by both techniques and 23 had more positive smears by TK2 than by TK1 (p < 0.001). Cytological examination of cytocentrifuge smears from the pool of BM samples after gradient density separation appears a simple, rapid and accurate technique for routine detection of BM involvement in neuroblastoma.     

Hypertrophic cardiomyopathy in mucopolysaccharidoses: Regression after bone marrow transplantation

Summary Mucopolysaccharide storage disease (MPS) presents clinically with a broad spectrum of abnormalities, among which cardiovascular involvement has been described.The echocardiographic findings have recently been reported for the various types of MPS. Among these, asymmetric septal hypertrophy (ASH) has been documented.We present a case of a 9-year-old girl suffering from type I MPS, atypical variant, with echocardiographic signs of ASH. She was given a bone marrow transplant after which the hypertrophic cardiomyopathy regressed.     

伴骨髓转移的神经母细胞瘤患儿初诊时骨髓染色体核型的临床意义

目的总结伴骨髓转移的神经母细胞瘤(NB)患儿初诊时骨髓染色体核型分析结果,探讨其临床意义。方法采用G显带的方法,对2015年1月至2017年12月北京儿童医院血液肿瘤中心收治的伴骨髓转移的NB患儿进行染色体核型分析,总结临床特点、分析预后,随访至2018年12月31日。结果1.共120例患儿,男74例,女46例,≥18个月者98例(81.7%)。染色体正常组60例,其中56例(93.3%)国际神经母细胞瘤分期系统(INSS)-Ⅳ期,余4例为INSS-Ⅳs期;低危(LR)2例,中危(MR)9例,高危(HR)49例(81.7%);7例患儿MYCN基因扩增。染色体异常组60例患儿均为INSS-Ⅳ期,MR 1例,余59例(98.3%)均为HR,14例MYCN基因扩增。2.染色体异常患儿60例中单纯数目异常和结构异常者分别为4例、14例,42例患儿同时合并染色体数目及结构异常。数目异常方面,21号、10号、11号染色体缺失最为常见;结构异常方面涉及11q、1p、3p区段的异常发生率高。3.染色体正常组患儿随访时间为4~44个月,17例出现肿瘤进展或复发;染色体异常组患儿随访时间2~42个月,其中31例出现肿瘤进展或复发。所有患儿3年累积总生存率和累积无事件生存率分别为60.0%、48.4%;染色体正常组患儿3年累积生存率为74.2%,3年累积无事件生存率为65.7%;染色体异常组患儿3年累积生存率为47.5%,3年累积无事件生存率为24.9%;出现进展或复发患儿染色体数目异常以10号染色体缺失常见,结构异常以11q、1p、2p区域较多。结论NB患儿肿瘤细胞染色体异常率高,但重复率低,个体间差异明显。10号染色体缺失、11q、1p、2p区域结构异常可能为提示NB预后不良因素;通过骨髓标本进行肿瘤染色体核型分析可行,可为更精准的危险度分层和治疗提供依据。     

Acute bone marrow necrosis caused by streptococcal infection

An 8 year old boy with a furuncle on the dorsum of the right foot, high fever, severe pain in the right knee joint, slight hepatosplenomegaly, leukopenia and thrombocytopenia was admitted with the working diagnosis of acute leukemia. However, an abundance of necrotic cells, together with clusters of streptococci, could be demonstrated in the bone marrow aspirate. After antibiotic therapy the boy recovered completely.

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Zusammenfassung Patientenbeobachtung mit Dokumentation des sehr seltenen Ereignisses der Knochenmarksnekrose durch bakterielle Toxine: Ein 8 Jahre alter Junge mit einem Furunkel am rechen Fußrücken, hohem Fieber, starken Knieschmerzen rechts, mäßiger Hepatosplenomegalie, Leukopenie und Thrombocytopenie wurde mit der Verdachtsdiagnose einer akuten Leukämie aufgenommen. Das Knochenmarkaspirat zeigte jedoch massenhaft nekrotische Zellen und viele, teils herdförmige Ansammlungen von kettenförmigen Bakterien. Nach antibiotischer Behandlung erholte sich der Junge völlig. Es wird eine kurze Literaturübersicht über das Krankheitsbild der Knochenmarksnekrose gegeben.

     

自体骨髓注射治疗单纯性骨囊肿的影像学变化与临床意义

目的观测自体骨髓注射治疗单纯性骨囊肿的影像学改变，分析其临床意义。方法自体骨髓注射治疗单纯性骨囊肿患儿27例，获随访25例，随访平均3．3年。以CR摄影判定影像学上的疗效；观测术后不同时期X线骨囊肿面积、皮质厚度的变化情况；分术前合并病理骨折组及无病理骨折组，测算术前囊肿纵径／最小皮质厚度，行组间比较。通过三维CT结合X线观察骨囊肿的基本愈合形式。结果1次注射后愈合10例，缺损愈合5例，持续存在7例（含2例无反应），复发3例。除外无反应者，术后3个月病灶内均有不同程度的密度增高；X线骨囊肿面积基本稳定，主要发生在术后649个月（21／23）；X线骨囊肿皮质厚度基本稳定，主要发生在术后9个月～1年（19／23）。除外下肢持重管状骨病变，术前合并病理骨折组及无病理骨折组囊肿纵径与最小皮质厚度比值差异显著。结论自体骨髓注射治疗单纯性骨囊肿术后随访至少1年；该法通过骨皮质增厚，囊腔内骨嵴及骨小梁向心性增生，分割包绕填塞囊腔而逐步愈合。     

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。     

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茵败血症。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。     

非血缘相关骨髓移植治疗重型地中海贫血的临床研究

目的为进一步拓展供髓源,探讨非血缘相关骨髓移植治疗重型地中海贫血(简称地贫)的可行性。方法9例地贫患儿进行了非血缘骨髓移植,其基因突变类型为地贫纯合子或双重杂合子,均确诊为重型β地贫。HLA高分辨配型全相合2例,1个亚型不合5例,2个亚型不合2例,6例红细胞血型不合。预处理为白消安16mg/kg,分4天口服;环磷酰胺200mg/kg,分4天静滴;抗人胸腺细胞免疫球蛋白30mg/kg,分3天静滴和氟达拉宾125mg/m2,分3天静滴。环孢素A和氨甲蝶呤预防移植物抗宿主病(graftversushostdisease,GVHD)。结果9例患儿均出现明显的过敏反应,1例有一过性低血压,皮肤急性GVHD7例,肝脏GVHD1例,肠道GVHD2例,慢性GVHD1例,高血压脑病1例,间质性肺炎2例。1例急性肺出血死亡。外周血中性粒细胞>0.5×109/L的时间为12～26天,WBC恢复正常的时间为23～110天,PLT于61～142天>50×109/L,Hb则于23～116天升至100g/L,最后一次输血时间为13～62天。PCR扩增短串联重复序列检测证实:8例患儿获得完全供体植入,1例未植入。随访6～24个月,7例原重型β地贫基因已消失,5例血型不合者已转成供者相同血型;7例患儿术后无需输血,Hb维持在110g/L以上。结论本组9例重型地贫的非血缘骨髓移植为国内首次尝试,初步证明非血缘骨髓移植根治重型地贫较安全可靠,为解决本病移植治疗的供髓源提供了新途径。     

Management strategies in foreign-body aspiration

Objective  The aim of this study is to outline a management algorithm to ensure effective teamwork in decreasing morbidity and mortality in pediatric Foreign-Body Aspirations (FBA). Furthermore, the role of flexible bronchoscopy when compared to rigid bronchoscopy in FBA was evaluated. Methods  Charts of patients with suspected FBA from October 1999 to September 2006 were reviewed and data with regards to the history, presenting symptoms, diagnostics and therapeutic tactics, was collected. Results  A total of 77 children with suspicion of FBA were managed in the 7 year period. Bronchoscopies were performed in 63 patients and in 26 foreign-bodies (FB) were found and extracted. At referral, 53 patients did not present acute respiratory symptoms, but had a positive history of FBA, and in 13 FB were found. Despite negative chest x-rays in 55 patients, FB were found in 8. Rigid bronchoscopy was performed in 53 and flexible in 10 patients. In 3 out of 10 patients who had undergone flexible bronchoscopy a FB was identified, the extraction of which was performed using a rigid bronchoscope. Conclusion  Clinical and radiological findings in children with typical history of suspected FBA are not enough to confirm the presence of FB. Successful management with an extremely low rate of morbidity and no mortality was observed using the algorithm used at our center. Flexible bronchoscopy reduces the chances of airway tract injury; however a rigid bronchoscope is necessary for FB removal.     

关节镜介入自体骨髓移植治疗单房性骨囊肿

目的：报道关节镜介入自体骨髓移植治疗15例单房性骨囊肿的疗效。方法：15例单房性骨囊肿患儿，男11例，女4例，平均年龄10岁1个月(7--18岁)。8例位于肱骨近端，5例位于股骨近端，1例位于股骨远端，1例位于胫骨近端。均在“C”型臂线机监视下骨囊肿置入关节镜，行囊肿壁搭刮处理后注入从髂骨吸取的骨髓。结果：15例患儿6个月后X线片示囊腔骨化，随访6～15个月骨愈合满意，未见并发症发生。结论：关节镜介入自体骨髓移植治疗单房性骨囊肿愈合快，疗效显著。