Cell-Mediated Immunity in Children with Hodgkin's and Non-Hodgkin's Lymphomas

Natural killer (NK) cell activity and lymphocyte proliferation to mitogens might have diagnostic implications that will help to distinguish between benign and malignant lymphadenopathy in children. In addition, we suggest that NK activity and especially the lowering of NK activity after chemotherapy might be of prognostic value in NHL children.     

Work-up and Diagnosis of Pediatric Non-Hodgkin's Lymphomas

Non-Hodgkin's lymphomas (NHL) in children and adolescents represent about 10% of childhood cancers. Although the types of NHL commonly seen in this population are relatively limited to lymphoblastic lymphomas, Burkitt's and Burkitt-like lymphomas, and large cell lymphomas, correct diagnosis and classification are essential for optimal therapy. Careful handling of pathologic specimens, along with collection of proper materials for ancillary studies such as immunophenotyping, cytogenetics, or molecular studies, will aid the pathologist in reaching a correct diagnosis. Specific morphologic, immunophenotypic, and genetic features of the commonly seen types of pediatric NHL are described.     

The Spectrum of Epstein-Barr Virus Hepatitis in Children

The clinical and pathologic features of Epstein-Barr virus (EBV) hepatitis in 3 children are described. Manifestations included fever, hepatomegaly, disseminated intravascular coagulation, and failure of uptake of technetium by the reticulo-endothelial system of the liver. Histologic features may mimic chronic active hepatitis and lymphoid malignancy. Two patients underwent exploratory laparotomy because of suspected tumor. Recognition of the wide spectrum of hepatic involvement in infectious mononucleosis is important in the differential diagnosis of hepatomegaly. Diagnosis should be made by measurement of IgM-specific EBV antibodies.     

Epstein-Barr Virus Infection and Serological Profile in Greenland Eskimo Children

ABSTRACT. The Epstein-Barr virus (EBV)-specific antibody profile of 101 Greenland Eskimo children was determined. The proportion of children with serological evidence of recent or past primary EBV infections rose from 22 % at 6 months of age to 79 % at 24 months of age. All but 2 of 49 children more than 4 years of age proved seropositive. The geometric mean titre (GMT) of antibodies to the viral capsid antigen (VCA) was highest during the first 3 years of life and declined sharply to a lower, nearly constant level in older children. The GMT of antibodies to the nuclear antigen (EBNA), rose slowly during the first 4 years of life to its persistent level. None of the children had a history of illnesses comparable to infectious mononucleosis. The results have shown that in this population with an enhanced risk of nasopharyngeal carcinoma, primary EBV infection occurs at a very early age.     

儿童EB病毒感染的免疫功能状况

目的研究EB病毒(EBV)感染儿童免疫功能状况及其与临床和预后的关系。方法用流式细胞仪(FCM)分别对30例传染性单核细胞增多症(IM)患儿急性期、恢复期外周血淋巴细胞CD3 、CD4 、CD8 、CD19 、CD23 抗原进行检测,并与20例同龄健康对照组儿童比较。结果IM患儿急性期CD4 、CD8 、CD19 、CD23 淋巴细胞百分率分别为(14.88±3.14)%,(65.49±5.33)%,(5.70±2.89)%,(2.41±1.83)%,恢复期为(36.75±3.88)%,(41.64±5.11)%,(15.98±2.80)%,(5.02±2.76)%。急性期CD8 明显高于恢复期和对照组,而CD4 、CD19 、CD23 淋巴细胞明显低于恢复期和对照组。恢复期3例CD23 细胞比值较高。结论EBV感染儿童外周血淋巴细胞亚群明显异常,CD23 细胞的持续增高与CD4 T细胞的长期低下有可能成为评价IM预后的指标。     

Primary Cutaneous Lymphomas in Children and Adolescents

Primary cutaneous lymphomas are rare in children and mostly represented by mycosis fungoides and CD30⁺ lymphoproliferative disorders. Most pediatric cutaneous lymphomas have similar clinical/pathological features as their adult counterparts, particularly the T‐cell subtypes. With regard to outcome, adult cutaneous mature T‐cell lymphomas have a tendency to progression, while this appears to be relatively infrequent in children. The outcome of cutaneous B‐cell lymphomas depends on subtype, with the B‐lymphoblastic entity being associated with similar outcomes to precursor B acute lymphoblastic leukemia, while there are insufficient data on other entities. The diagnosis and treatment of these patients require a close collaboration between experienced pediatric pathologists, dermatologists, and oncologists. Prospective collection of longitudinal clinical and biological data from children with these rare lymphomas is needed to better understand their biological and clinical behavior and to ultimately discover the best therapeutic strategies.     

急性淋巴细胞白血病并EB病毒感染的临床分析

目的 探讨EB病毒(EBV)在急性淋巴细胞白血病(ALL)儿童中的感染及其临床意义.方法采用荧光定量聚合酶链反应(FQ-PCR)技术检测EBV DNA,ELISA法检测EB病毒衣壳抗原IgM抗体(EBV-CA-IgM),共检测47例.其中新发45例,复发2例;年龄0～14岁[(8.06±3.71)岁].另取14例健康儿童作为健康对照组.男9例,女5例;年龄2～10岁[(7.24±2.54)岁].结合临床表现、诱导治疗骨髓完全缓解(CR)率、形态学CR状态下的微小残留病(MRD)、复发率及无事件生存(EFS)率等分析ALL患儿中EBV感染情况及其临床意义.结果 47例ALL患儿中检出EBV感染15例(31.9%),其中11例(23.40%)检出EBV DNA,EBV DNA水平为(3.28±5.95)×108copy·L-1;14例健康对照组外周血未检测到EBV DNA及EBV-CA-IgM.ALL中EBV感染组与非EBV感染组白细胞数分别为(78.00±58.38)×109 L-1、(27.46±60.10)×109 L-1(t=2.70,P=0.01),诱导治疗CR率分别为 46.7%、87.5%(P<0.01),MRD>10-3分别为90.0%、26.1%(P<0.01),复发率分别为53.8%、13.8%(P<0.01),EFS率分别为 23.1%、82.8%(P<0.01).结论 ALL并EBV感染具有高白细胞数、低诱导治疗CR率、高复发率、低EFS率,提示EBV感染可能参与儿童ALL的发生发展过程,亟待改善EBV感染ALL的治疗方法.     

儿童EB病毒传染性单核细胞增多症临床特征及诊断标准

传染性单核细胞增多症(IM)是儿童原发性EB病毒感染的典型表现。国外的诊断标准不适合我国儿童EB病毒IM的诊断。本文介绍儿童EB病毒IM的临床特征、适合我国儿童EB病毒IM的诊断标准及处理原则。     

Kawasaki Disease and Epstein-Barr Virus

We report the results of virological (serological and molecular biological) studies of Epstein-Barr virus (EBV) in patients with Kawasaki disease (KD). Forty-nine (86%) of 57 Kawasaki disease patients and 15 (68%) of 22 patients with recurrent Kawasaki disease had serological evidence of primary Epstein-Barr virus infection during the first month after the onset of their disease based on the results of a sensitive method of detecting antibody to viral capsid antigen (VCA). The serological response to EBV was significantly low and transient. EBV sequences were identified directly in peripheral blood mononuclear cell (PBMC) DNA samples from 23 (56%) of 41 KD patients within 2 weeks after onset by means of the polymerase chain reaction (PCR). EBV sequences were also detected in 10 (83%) of 12 repeatedly tested KD patients within 3 months after onset. In contrast, only 7 (18%) of 40 control DNA samples were PCR-positive. These virological studies indicate that an unusual EBV-cell interaction may occur in KD.     

儿童NK/T细胞淋巴瘤的免疫表型及其与EB病毒的关系

目的探讨儿童NK/T细胞淋巴瘤的免疫表型特征及其与EB病毒（EBV）感染的关系。方法NK/T细胞淋巴瘤5例标本,采用免疫组织化学链霉素抗生物素-过氧化酶连接法（SP法）检测CD45RO、CD3ε、CD56、CD20、T细胞内抗原（TIA-1）、丝氨酸蛋白酶-粒酶B（Granzyme B）确定肿瘤细胞免疫表型及EBV潜伏膜蛋白（LMP-1）,原位杂交技术检测EBV编码的RNA（EBER1/2）。结果NK/T细胞淋巴瘤5例CD45RO、CD3ε、TIA-1和Granzyme B全部阳性,CD56阳性2例,CD20全部阴性。EBER1/2阳性4例,LMP-1阳性3例。结论EBV感染与儿童NK/T细胞淋巴瘤关系密切,EBV感染在儿童NK/T细胞淋巴瘤的发生发展中可能起重要作用。     

EB病毒感染及其相关性疾病

EB病毒(EBV)感染是儿科较为常见的病毒感染性疾病,不同的免疫状态有不同的临床表现。机体感染EBV后,可以产生传染性单核细胞增多症、EBV相关性噬血细胞综合征、慢性活动性EBV感染、X连锁淋巴组织增生性疾病及鼻咽癌、淋巴瘤等。免疫缺陷患者感染EBV后容易发生淋巴组织增殖性疾病。传染性单核细胞增多症是EBV感染最常见的临床表现形式,常合并一系列的并发症。     

EB病毒感染与淋巴增殖性疾病

EB病毒(EBV)是一种能诱发肿瘤的疱疹病毒.多数情况下,由于机体的免疫系统能严格限制病毒颗粒的增殖,受感染者终生携带病毒而不发病.少数情况下如机体免疫功能明显降低,则可引起严重的相关性疾病,特别是良性或恶性的淋巴增殖性疾病.近年来,有关EBV在人体内生存的机制、感染后的免疫调控、相关疾病的发病机制等方面均有了新的认识.现就近年来EBV与淋巴增殖性疾病的有关进展情况进行介绍.     

EB病毒感染及其相关性疾病

EB病毒（EBV）感染是儿科较为常见的病毒感染性疾病，不同的免疫状态有不同的临床表现。机体感染EBV后，可以产生传染性单核细胞增多症、EBV相关性噬血细胞综合征、慢性活动性EBV感染、X连锁淋巴组织增生性疾病及鼻咽癌、淋巴瘤等。免疫缺陷患者感染EBV后容易发生淋巴组织增殖性疾病。传染性单核细胞增多症是EBV感染最常见的临床表现形式，常合并一系列的并发症。     

Thymic Mucosa-associated Lymphoid Tissue Lymphoma in an Adolescent Girl

Mucosa-associated lymphoid tissue (MALT) lymphoma is very rare in children. We report the first case of pediatric thymic MALT lymphoma in an adolescent Asian girl. She presented with chest pain, dyspnea, and low-grade fever. A large anterior mediastinal mass was biopsied that confirmed the diagnosis of MALT lymphoma with trisomy 18. The patient had secondary immunodeficiency with low NK cell count and high IgA and IgG levels. Because of the advanced stage and the presence of trisomy 18, she was treated with cyclophosphamide, vincristine, prednisone, and rituximab, followed by involved-field radiotherapy. She is currently undergoing maintenance therapy with rituximab and remains in complete remission at 13 months from diagnosis. Thymic MALT lymphoma should be suspected in any Asian child with a cystic thymic mass and autoimmune disease or hyperglobinemia. Because of the slow proliferation rate of this type of lymphoma, a long-term follow-up is needed.     

儿童嗜酸性淋巴肉芽肿的临床特点与治疗

目的 探讨儿童嗜酸性淋巴肉芽肿（ELG）的临床特点和诊治方法，以提高对本病的认识。方法 回顾性分析5例ELG患儿的临床特征、实验室检查、病理、治疗及疗效，并进行相关文献复习。结果 ELG5例均为男童，主要表现为多发性皮肤软组织肿块和淋巴结大，其中1例并肾病综合征，2例有反复湿疹样皮疹，1例2次发生喘息性支气管炎，患儿均无肝脾大，生长发育良好。最初单纯化疗4例均复发，经配合局部放疗或加用环孢素（CsA）等治疗后未复发。结论 儿童ELG临床及病理特点与成人相似，但儿童发病较少。单纯化疗易复发，配合放疗、手术、应用CsA等综合治疗可提高疗效。     

Significance of Lymphoid Follicles and Aggregates in Gastric Mucosa of Children

This study was designed to evaluate the significance of gastric lymphoid follicles (LF) and aggregates (LA) in children with and without Helicobacter pylori (HP) infection. All 605 antrum biopsies performed during 1994 were reviewed and classified according to the presence or absence of inflammation, LF, or LA. HP was searched with a DiffQuik stain in all biopsies showing gastritis, LF, or LA. Gastritis was diagnosed in 80 biopsies (16 with LF, 18 with LA and 46 without LA or LF). Identification of HP in these biopsies was as follows: (a) cases with LF: 12/16; (b) cases with LA: 3/18; (c) cases without LF or LA: 8/46. The biopsies without gastritis had a higher frequency of LA (65/525) than of LF (2/525). HP was not identified in any case without gastritis. The presence of LF with histologic gastritis had the strongest correlation with HP (R = 0.5, p < 0.00001). LF with gastritis had a positive predictive value of 75% for HP and the absence of LF had a negative predictive value of 82.8% (sensitivity 52%; specificity 92%). LA with gastritis had no significant correlation with HP. From these results we conclude that lymphoid follicles should be distinguished from lymphoid aggregates. Lymphoid follicles can rarely be present in an otherwise normal gastric mucosa; however, they are more frequently found in cases of gastritis and are strongly associated with HP infection. Lymphoid aggregates are not significantly associated with HP infection and may be a component of the normal gastric lymphoid tissue. Received March 9, 1999; accepted May 12, 1999.