儿童脾错构瘤并多系统免疫性疾病1例  

Splenic Hamartoma with Immune Disorders in Multiple Systems in One Child

作　　者：陈曦 王淑红 张静 

机构地区：[1]乌鲁木齐儿童医院神经内科,乌鲁木齐830002 [2]乌鲁木齐儿童医院血液科,乌鲁木齐830002

出　　处：《实用儿科临床杂志》2012年第24期1883-1885,1902,共4页Journal of Applied Clinical Pediatrics

摘　　要：目的通过对一典型儿童脾错构瘤(SH)病例临床特征、组织病理和脾全切除术后转归的观察,提出SH并多系统免疫疾患的可能性。方法总结1例SH患儿临床资料。血常规、骨髓、Coombs检查及红细胞脆性试验确认自身免疫性溶血性贫血(AIHA)的诊断,对被切除的脾肿瘤进行组织病理学和脾瘤特异性免疫组织化学检测,以确认SH诊断,急性惊厥期行常规脑脊液检查、头颅MRI和视频脑电图(VEEG)检测,以支持其脱髓鞘性脑炎诊断,行脾和SH全切除术后随访2.5 a,以确认相关并发症的远期转归。结果患儿出生3个月反复出现黄疸、贫血伴进行性脾大。12岁入本院。查体:肝右肋下4 cm,脾重度大。血常规示重度贫血,网织红细胞0.147。Coombs直接试验阴性,但间接试验阳性。骨髓检查示骨髓增生活跃,红系增生显著异常。红细胞渗透脆性显著增高,AIHA诊断成立。腹部增强CT示脾实质有一7.5 cm×8.0 cm类圆形孤立肿块,脾瘤病理学切片见结构紊乱脾红髓,免疫组织化学检查示瘤内所有细胞呈低增殖指数,增殖抗原抗体Ki-67<5%。其中血管内皮细胞呈显著CD8(+)反应,散在或灶性分布。同时血管间隙见到散在Ⅷ因子(+)、波形蛋白(+)、CD34(+)、CD31(+)和CD68(+)细胞,确认SH诊断。入院第11天突发反复惊厥伴意识障碍,VEEG示全脑弥散性异常δ活动,头颅MRI示双侧侧脑室前后角和右侧枕叶白质区斑片状信号,脑脊液常规及生化检查正常,但髓鞘碱性蛋白检测阳性,符合脱髓鞘性脑炎。入院第25天行脾和SH全切除术。术后停用所有药物,并随访2.5 a,未再有溶血及惊厥发作;术后0.5 a复查:生长发育明显改善,血常规、Coombs和网织红细胞计数正常;术后1 a VEEG及脑MRI均恢复正常。结论儿童SH较成人更易并各种血细胞或血小板减少,但长期被认为是由SH引发的脾功能亢进所致。本例为世界第2例报道,但Coombs间接试验阳性SH儿童并AIHA�Objective To suggest the new ideas on the possibility that splenic hamartoma（SH） in children could be complicated with immune disorders in multiple systems on account of a novel case with typical clinical and histopathology presentation, as well his satisfied out- come after splenectomy. Methods Clinic data of 1 child with SH were collected. The diagnosis of autoimmune hemolytic anemia （AIHA） was made according to routine tests of blood, bone -marrow, Coombs, and red blood cell （RBC） fragility tests. The diagnosis of SH was made after splenectomy with histopathology and the specific immunohistochemistry stains for splenic tumors. The cerebrospinal fluid （CSF） routine test, cranial magnetic resonance imaging（MRI） and video electroencephalogram（VEEG） studies were performed during the activity stage of con- vulsion and the diagnosis of demyelination encephalitis was supported. The remote outcome for the all related complications was confirmed in 2.5 years follow - up after the surgical remove of spleen and SH. Results The patient suffered from recurrent episodes of jaundice, anemia complicated with chronic and progress splenomegaly at 3 months after birth. The inferior margin of the magnified liver was found at the 4 cm under the right ribs region and severe splenomegaly was presented at the age of 12 years when he was admitted to Children＇s Hospital of Urum- gi. Routine blood analysis showed severe anemia with elevated reticular RBCs（0. 147）. Direct Coombs test was negative,but indirect test was positive. Bone marrow test showed active proliferation in bone marrow but abnormal RBC genesis obviously, and elevated RBC osmotic fragili- ty,confirming the diagnosis of AIHA. A single, circular like tumor （7.5 cm × 8.0 cm） was detected with contrast CT. Histologic findings con- sist of unorganized vascular channels with intervening red pulp - like disorganized stroma, hnmunohistochemistry on the lining cells of the splenoma vascular had a low proliferation index due to the antibody Ki -6

关 键 词：脾错构瘤 自身免疫性溶血性贫血 自身免疫学紊乱 

分 类 号：R733.2[医药卫生—肿瘤] R725.9[医药卫生—临床医学]