Graft versus host disease and cytokines

Allogeneic hematopoietic stem cell transplantation(Allo-HSCT) is a standard therapy for hematological malignancies. However, graft versus host disease(GVHD) remains one of the major obstacles for successful Allo-HSCT. The insight into the pathogenesis of GVHD has been profoundly improved by recent advances in our understanding of the immune system and progress of the molecular biology. A primary causes for GVHD are the inappropriate production of cytokines and dysregulation of complex cytokine network occurs in the three sequential steps: conditioning regimen, donor T cell activation and effector mechanisms. This short review discusses the interaction between the cytokines and the cellular effector of GVHD in each step.     

小儿寄生胎1例

患儿男,6岁,主因"发现腹部包块3天"入院.查体:左中下腹触及一类圆形包块,大小约5 cm×5 cm×5 cm,表面尚光滑,边界清楚,活动度良好,无压痛,无波动感,肝、胆囊、脾肋下未触及,Murphy征阴性.左侧阴囊空虚,未触及睾丸,右侧阴囊内可触及实质性睾丸,大小、质地中等.     

Photopheresis of a less than 10-kg child with acute graft versus host disease accompanied with hyperbilirubinemia: A case report

Hematopoietic stem cell transplantation is a curative treatment for many hematologic malignancies with its most important side effect being graft-versus-host disease (GVHD). Herein, we present a 3.5 year-old male with weight of 9.8?kg with acute GVHD (grade IV gastrointestinal and cutaneous) who did not respond to the first line therapies (corticosteroids). Thus, the patient was a candidate for extracorporeal photochemotherapy (ECP). Due to the hyperbilirubinemia, two sessions of ECP every week as well as one session of plasmapheresis 24?h before each ECP session were performed (Spectra^?Optia^® apheresis system). The procedures were performed successfully without any side effects and the GVHD manifestations of skin and GI responded perfectly to the treatment after 12 and 14 sessions of ECP, respectively. According to the results, it seems that ECP could be successfully performed in even less than 10-kg patients.     

异体脐血干细胞移植导致胃肠移植物抗宿主病的实验研究

目的观察心肌梗死(MI)大鼠接受静脉异体脐血干细胞(HUCBC)移植后发生腹胀的原因。方法大鼠于心肌梗死后5 d经尾静脉注入0.5 ml脐血[含单个核细胞(1～3)×107]或0.5 ml磷酸缓冲盐溶液(PBS)。不使用免疫抑制剂。观察8周,入选移植后发生腹胀的大鼠、未腹胀大鼠、对照(PBS)组大鼠(每组n=8),行超声心动图和血流动力学检查,同时对扩张肠道进行HE染色明确腹胀原因。结果梗死后移植大鼠心功能明显优于PBS大鼠,而腹胀与非腹胀大鼠相比,射血分数(EF)、短轴缩短率(FS)、左室压力最大变化率(±dp/dtmax)、左室舒张末压(LVEDP)没有显著差异,因此腹胀的发生与心功能恶化及移植效果没有关系。肠道大体解剖和病理分析显示移植大鼠(包括腹胀和未腹胀大鼠)均存在肠道扩张,以回肠周围最为明显,组织学表现符合肠道移植物抗宿主病。结论异体脐血单个核细胞移植可以导致胃肠道移植物抗宿主反应。     

Photopheresis long after the initiation of chronic graft versus host in a child

Extracorporeal photopheresis (ECP) has been used widely in the treatment of steroid-refractory chronic graft versus host disease (cGVHD). Several reports have applied an ‘early treatment’ approach due to the better response rates compared with late treatment. However, herein, we report a hematopoetic stem cell transplantation performed in a thalassemia major patient presenting with severe cGVHD who applied to our center for ECP treatment nearly 12 years after the onset of cGVHD.     

小儿胚胎型横纹肌肉瘤1例

患儿男,2岁,主因"发现左腋下肿物1个月"人院.查体:左腋下可触及一个8 cm×5 cm肿块,延至左肩胛下缘及左侧胸部.超声:左腋下、左肩胛下及左侧胸壁皮下探及一个以实性为主的囊实性团块,其腋下深度为4.72 cm,肩胛骨处深度4.55 cm,左侧胸壁皮下深度3.95 cm,病灶边界不清,形态不规则,内部回声不均匀,其内可见低回声区及点状强回声;CDFI:病灶内可见动、静脉血流信号(图1).CT:双肺见多发结节,气管隆嵴下及左肺门淋巴结肿大.影像学提示:左腋下恶性占位性病变伴双肺及肺门、纵隔淋巴结多发转移.穿刺活检诊断为胚胎型横纹肌肉瘤.予长春新碱及放线菌素化疗后肿物明显缩小,予以手术切除,术中见肿瘤约7 cm×6 cm,有包膜,血运丰富.术后病理检查证实为胚胎型横纹肌肉瘤.     

儿童脑干伴多层菊形团形成的胚胎性肿瘤1例

<正>女性患儿，3岁，走路不稳3天，既往体健。查体：神志清，反应差，颈软无抵抗，四肢肌力、肌张力减弱。头颅CT：脑干左侧稍高密度为主囊实性病灶，边界清晰，鞍上池受压变形。头颅MRI：中脑及脑桥左侧31mm×38mm×31mm肿块，边界清晰，呈T1WI低信号、T2WI稍高信号（图1A）,T2液体衰减反转恢复序列呈等低信号，弥散加权成像（diffusion weighted imaging,DWI）部分受限（图1B），表观弥散系数（apparent diffusion coefficient,ADC）图呈低信号（图1C）,     

儿童原发性椎管内髓外硬膜下尤因肉瘤1例北大核心CSCD

女性患儿,13岁,3天前跌伤后感腰痛,活动时加重,休息后可缓解;既往体健,无特殊家族病史。查体:L2~L4椎体棘突间隙处及椎旁软组织轻度压痛,余未见明确异常。实验室检查:D-二聚体3588μg/L。腰椎CT:L2~L3椎管内见12 mm×9 mm×22 mm等密度软组织肿块影,边界欠清,CT值约51 HU,邻近骨质未见破坏。腰椎MRI:L2~L3水平椎管内髓外硬膜下10 mm×8 mm×20 mm肿块,边界清,T1WI、T2WI呈均匀等信号(图1A)。     

Case report: Laparoscopic excision of urachal cyst in a child

Summary

A urachal cyst in a 13-year-old boy presented with symptoms of abdominal pain and microscopic haematuria was removed laparoscopically. The case is presented and the procedure is discussed.     

儿童管状型肠重复畸形伴肠旋转不良1例

本文介绍1例较长的管状型儿童肠重复畸形并伴肠旋转不良，其在诊断过程中各检查方法的不同表现。