论文部分内容阅读
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尚属世界首例。高度提示SH有并AIHA的可能性,对原因不明的AIHA应除外SH的可能。目前尚不能确认本例脑炎与SH有肯定关联,但儿童SH是否存在更多系统免疫异常值得进一步关注。
Objective To investigate the clinical characteristics, histopathology and outcome after splenectomy in a typical childhood spleen hamartoma (SH) and to propose the possibility of SH and multiple system immune disorders. Methods One case of SH children clinical data. Blood routine, bone marrow, Coombs test and erythrocyte fragility test confirm diagnosis of autoimmune hemolytic anemia (AIHA), histopathology and spleen-specific immunohistochemical detection of excised spleen tumor to confirm SH diagnosis, acute Convulsions routine cerebrospinal fluid examination, cranial MRI and video EEG (VEEG) testing to support its demyelinating encephalitis diagnosis, splenic and SH total resection follow-up of 2.5 a, to confirm the long-term complications Go. Results 3 months after birth, children with recurrent jaundice, anemia with progressive splenomegaly. 12 years into the hospital. Physical examination: liver right rib 4 cm, splenomegaly. Blood showed severe anemia, reticulocytes 0.147. Coombs Direct test negative, but the indirect test is positive. Bone marrow examination showed active bone marrow hyperplasia, erythroid hyperplasia significant abnormalities. Erythrocyte infiltration fragility increased significantly, AIHA diagnosis established. Abdominal enhanced CT showed splenic parenchyma with a 7.5 cm × 8.0 cm round isolated lumps, pathological section of spleen see spleen red pulp structure disorders, immunohistochemical examination showed all cells in the tumor was low proliferation index, proliferation antigen antibody Ki-67 <5%. Vascular endothelial cells showed significant CD8 (+) reaction, scattered or focal distribution. At the same time see the vascular interstitial scattered Ⅷ factor (+), vimentin (+), CD34 (+), CD31 (+) and CD68 (+) cells, confirm SH diagnosis. Recurrent seizures with unconsciousness on the 11th day of admission, VEEG showed diffuse abnormalities of whole-brain activity, cranial MRI showed anterior and posterior horn of the bilateral lateral ventricles and white matter patchy occipital lobe of the right cerebrospinal fluid routine and biochemical tests were normal, But myelin basic protein test positive, in line with demyelinating encephalitis. Admission on the 25th day spleen and SH total resection. All drugs were discontinued after operation and were followed up for 2.5 years. No further hemolysis or seizures occurred. After 0.5 years of follow-up, the growth and development were significantly improved. The blood routine, Coombs and reticulocyte count were normal. VEEG and MRI All returned to normal. Conclusion Childhood SH is more susceptible to various kinds of blood cells or thrombocytopenia than adults, but SH is thought to be caused by hypersplenism over a long period of time. This case is the world’s second case report, but Coombs indirect test positive SH children and AIHA is the world’s first case. It is highly suggestive of the possibility that SH has AIHA and SHHA may be excluded for unexplained AIHA. It is not yet confirmed that there is a positive correlation between encephalitis and SH in this case, but whether there is more systemic immune abnormality in children SH deserves further attention.