皮下脂膜炎样T细胞淋巴瘤一例报道并文献复习

通过对我院一确诊为皮下脂膜炎样T细胞淋巴瘤(SPTL)病例的分析,及对相关文献的复习,进一步对SPTL的流行病学、组织病理学、免疫组织化学特点,治疗进展及预后等方面进行总结,以期提高对这一疾病的认识.SPTL为儿科少见疾病.组织病理学检查主要表现为异形淋巴细胞围绕在脂肪细胞周围,呈典型"花边样"浸润,偶见组织细胞及吞噬细胞,吞噬核碎片的组织细胞呈"豆袋样".免疫组织化学染色常为CD4~/CD8~+,偶可CD56~+,无论其表达何种T细胞分化抗原,均应表达细胞毒颗粒相关蛋白(TLA-1、GB和perforin)和βF,并可表达LCA和CD45RO.CHOP或类似CHOP的方案、环孢素A、fludarabine(氟达拉滨)、denileulin diftitox和bexarotene(蓓萨罗丁)在治疗SPTL中已取得一定疗效.关于本病预后的研究不多,合并噬血细胞综合征、血管浸润可能是预后不良的信号.     

小儿脂膜炎样T细胞淋巴瘤一例

患儿女 ,1岁 10个月。因全身皮下结节、反复皮疹 1个月 ,鼻塞、双眼睑水肿 7天入院。病后精神、饮食差 ,无发热、咳嗽及流涕 ,曾在外院按“上呼吸道感染”等治疗无效。体检 :T 37℃ ,R 34次 /min ,P 10 0次 /min ,体重 12kg。一般情况差 ,慢性病容 ,轻度贫血貌 ,无呼吸困难 ,颜面略肿 ,双眼睑明显水肿 ,双眼球微突 ,口腔黏膜附着白色斑膜状物、左眉部、躯干、臀部、会阴部皮下有约 0 5cm× 0 5cm至1 0cm× 1 0cm大小的结节 ,以腹部较多 ,呈暗紫色 ,表面光滑 ,皮温不高 ,压之褪色 ,质中等 ,活动度差 ,无压痛。颈部、躯干可见成簇分布的米…     

儿童皮下脂膜炎样T细胞淋巴瘤1例报道并文献复习

目的 分析儿童皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床表现及病理组织学特征,探讨其病理诊断及鉴别诊断,提高儿科医师对该病的认识.方法 反复发热伴多发性皮肤损害、病程长达2 a确诊的SPTCL患儿1例,对其临床表现的演变、多次被误诊的就医经历、确诊时的组织病理学特点、免疫组织化学结果及T细胞受体(TCR)基因克隆重排等方面进行观察,并对相关文献进行复习.结果皮肤活检发现组织学病变主要局限在皮下脂肪组织内,非典型淋巴细胞围绕单个脂肪细胞形成特征性的花边样结构.免疫组织化学显示CD45、CD45RO、CD3、CD5、T细胞内抗原-l及穿孔素呈阳性表达,而CD10、CD20、CD56、CD68、上皮膜抗原和细胞角蛋白均呈阴性,提示瘤细胞为T细胞表型.TCR基因克隆重排检测:免疫球蛋白H基因重排FR2(+),FR3A(-);TCRr基因重排JVI(-),JVII(+);TCRB基因重排JD1(-),JD2(-).按照儿童T细胞非霍奇金淋巴瘤方案治疗,但疗效欠佳.结论SPTCL是一类特殊类型的原发性皮肤淋巴瘤,预后较差,对不明原因的反复发热伴皮肤多发性损害的儿童,病损处皮肤活检是确诊SPTCL的重要手段.     

以眶内肿物为主要表现的儿童皮下脂膜炎样T细胞淋巴瘤一例

患儿男 ,9岁。因反复皮肤结节 2年余 ,右眼眶肿物伴会阴部红肿溃烂 1个月入院。入院前 2年余患儿躯干、四肢皮肤反复出现紫红色黄豆大结节 ,不疼不痒可自行消退。入院前 1个月发现颜面及会阴皮肤出现红色斑块 ,右眼睑外眦部出现一个花生米大小肿物并进行性增大 ,伴红肿触痛。入院前 1个月在当地医院行“包皮环切术” ,术后切口周围及阴囊红肿 ,继之破溃形成溃疡。体检 :体温 37 5℃ ,脉搏 12 0次 /ｍｉｎ ,呼吸 2 8次 /ｍｉｎ ,血压 12 0 / 75ｍｍＨｇ。颜面皮肤可见多处红色条纹及斑块 ,双眼球结膜及睑结膜充血 ,右下眼睑外眦部可见一 2 5…     

皮下脂膜炎样T细胞增生症1例

1病例报告 患儿，男，10岁，因反复发热20个月，发现肝脾大9个月入院。患儿于入院前20月开始发热，热峰39．5℃，一般午后发热傍晚缓解或夜里发热清晨缓解，纳差，无畏寒，无大汗，无咳嗽，无腹痛腹泻，无贫血出血。入院前9个月在当地医院发现肝脾大，在多家医院查血培养、肥达反应、疟原虫、血吸虫抗体、[第一段]     

皮下脂膜炎样T细胞淋巴瘤一例

患儿 女,14岁,2004年8月23日入院.因多发皮下结节1个月余,发热13 d入院.人院前1个月全身相继出现多处同样皮下结节.13 d前开始发热,体温39℃以上,发热时肿块有肿胀感.腹软,肝脾不大,未及包块.右面颊、右颈、躯干、四肢见多处1～3 cm大小皮下结节,质地稍硬,无触痛,表面皮肤淡红色或暗红色.     

儿童皮下脂膜炎样T细胞淋巴瘤1例病例报告

正1病例资料女,9岁,2016年4月2日因"发热1月余伴臀部软组织肿胀"于上海市儿童医院(我院)住院治疗。患儿的重要临床信息见图1。患儿1个月前出现发热,呈持续高热,热峰39℃,并出现臀部软组织肿胀,皮肤呈青紫状,肿胀范围渐扩大,中间皮肤破溃,无渗液,无触痛,无髋关节活动障碍。外院曾予阿米卡星、磷霉素、病毒唑和地塞米松等,仍发热,臀部皮损扩大。患儿既往体健,生长发育正常,否认家族遗传病史。     

婴儿结节性脂膜炎演变为儿童皮下脂膜炎样T细胞淋巴瘤1例报告

正病例资料1病情简介患儿,男,9岁,以"发热伴双颊肿痛14 d,加重4 d"入院。患儿于入院14 d前出现发热,伴双颊部肿痛,局部皮肤无发红,尚可忍受,4 d前症状加重,伴局部皮肤发红,热峰升高,体温最高升至39.3℃,每日热峰2~3次,伴畏冷、寒战,无咳嗽、咳痰,无咽痛,无腹痛、腹泻,无皮疹,遂转诊我院。既往史:患儿出生后6个月时因"反复发热、皮疹、躯干及左髂骨处皮下肿物"就诊外院,行左髂骨     

Pediatric Subcutaneous Panniculitis-Like T-Cell Lymphoma with Favorable Result by Immunosupressive Therapy: A Report of Two Cases

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare type of skin lymphoma. Histopathology mimicking a lobular panniculitis makes it difficult to distinguish SPTL from benign autoimmune disease. We present cases of a 10-year-old female and an 11-year-old male with SPTL showing recurrent panniculitis and systemic manifestations. Initially, antibiotics and steroids were administered to treat infectious disease and benign panniculitis. However, they experienced recurrent fever and erythema nodosum. Additional immunohistochemistry and T-cell receptor (TCR) gene rearrangement analyses were performed, enabling the establishment of an SPTL diagnosis. The affected patients were given immunosuppressive therapy with favorable results.     

骨原发性恶性淋巴瘤1例

患儿,男,12岁,3个月前不慎摔伤右手,当即感右肘部疼痛、迅速肿胀,活动受限,诊断为右肱骨骨折。行手法复位后,石膏夹板固定,1个月后自行拆夹板,约20 d后骨折处局部肿胀并渐加重,10 d前肘部开始疼痛并渐加重。故来我院就诊。门诊以右肱骨远端恶性肿瘤收住院。查体:体温36℃,脉搏7     

Subcutaneous t-cell Lymphoma Presenting as Panniculitis in Children: Report of Two Cases

Two cases of peripheral T-cell lymphoma with primarily subcutaneous involvement and clinically presenting as panniculitis were reported in two children. One child developed florid hemophagocytic syndrome and was treated by combination chemotherapy but died 14 months later of disseminated fungal infection. The other child had mild systemic symptoms but no histological evidence of hemophagocytosis. Despite a more florid lymphomatous infiltrate, he attained a complete remission after a short course of prednisone and remains symptom-free at 3 years. Our report confirms that subcutaneous T-cell lymphoma is a distinct clinicopathological entity and highlights the two modes of clinical presentation. The development of florid hemophagocytic syndrome indicates a bad prognosis. This entity must be distinguished from other causes of panniculitis and immunophenotypic analysis of the atypical cells is essential in the diagnosis. Combination chemotherapy is the treatment of choice, but in patients who pursue an indolent course the administration of aggressive chemotherapy may be deferred.     

Nijmegen Breakage Syndrome-associated T-cell-rich B-cell Lymphoma: Case Report

In 1981 Weemaes et al. first described the Nijmegen breakage syndrome (NBS), a rare autosomal recessive disorder characterized by stunted growth, microcephaly, immunodeficiency, spontaneous chromosome instability, and a peculiar predisposition to cancer development. Most NBS-related malignancies are lymphomas, but their pathologic features have rarely been specified. We report here the case of a northern Italian 8-year-old child who, 2 years after the diagnosis of NBS, developed a diffuse large B-cell lymphoma (T cell–rich B-cell lymphoma variant). The histological and immunobiological features of the lymphoma population are analyzed and discussed in detail. Received March 29, 1999; accepted June 18, 1999.     

Pediatric Anaplastic Lymphoma Kinase-Positive Large B-cell Lymphoma: A Case Report and Review of the Literature

ABSTRACT We present a case of pediatric anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK-positive LBCL) with a cervical, mesentery, and pelvis cavity mass. Histologic examination of the cervical mass revealed that the lesion was composed of diffuse large immunoblastic-like or plasmablastic-like tumor cells with a sinusoidal growth pattern. The tumor cells were strongly immunoreactive for ALK; revealed a granular cytoplasmic distribution; and were diffusely positive for CD45, CD4, CD138, epithelial membrane antigen, and multiple myeloma oncogene-1 but negative for CD20 and CD79a. The patient underwent 5 courses of cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposid and obtained a remarkable clinical response with regression of mesentery and pelvis cavity mass. We suggest that this distinct subtype of large B-cell lymphoma should belong to the spectrum of pediatric lymphomas and that radiologic examination should be performed to inspect the progression of disease even if the patients experienced complete remission at initial chemotherapy.     

MALT Lymphoma in Children: Case Report and Review of the Literature

Mucosa-associated lymphoid tissue (MALT) lymphoma predominantly occurs in adults, and is rare in children. We report a case of MALT lymphoma involving minor salivary gland of the lip in an otherwise healthy 12-year-old boy. This is the second case report of MALT lymphoma of minor salivary gland in an immunocompetent child. Of 24 cases of MALT lymphomas in children reported in the English literature, parotid MALT lymphomas in human immunodeficiency virus (HIV) patients and H. pylori infection-associated gastric MALT lymphomas are the most common. As in adult cases, most MALT lymphomas in the pediatric age group are localized and follow an indolent clinical course, respond well to therapy, and have an excellent outcome.     

Non-Hodgkin Lymphoma in a Child Presenting with Acute Paraplegia: A Case Report

Spinal cord compression is a rare presentation of non-Hodgkin lymphoma. Extradural location at onset is a rare but devastating event in pediatric oncology. The authors describe a girl with acute spinal cord compression due to epidural non-Hodgkin lymphoma, emphasizing the encouraging perspective for a complete recovery in children with this condition. A 5-year-old girl presented with pain followed by progressive hyposthenia and paraplegia after a trauma. CT scan and MRI showed homogeneous tissue extending from T2 to L4, occupying the entire vertebral canal and extending to the para- and peri-vertebral soft parts. Emergency surgical debulking was carried out through T6-L1 laminectomy. The patient began chemotherapy (LMB 89 Protocol) and the tumor quickly disappeared. The patient is maintaining a complete remission 42 months after diagnosis. Significant results may be obtained with the chemotherapy treatment of epidural non-Hodgkin lymphoma when the disease is promptly diagnosed. Considering the effectiveness of chemotherapy, the authors believe that a neuro-surgical approach should be employed only when rapid worsening of symptoms is observed or for diagnostic purpose.