Early diagnosis of intestinal non-Hodgkin lymphoma by endoscopy

The early diagnosis of intestinal non-Hodgkin lymphoma, stages I and II, lymphoblastic of Burkitt's type, was made by colonoscopy combined with selective radiologic visualization of the tumor in two children, ages 4 and 5. This technique is recommended in patients with abdominal pain, iron-deficiency anemia, intestinal blood loss, and an elevated sedimentation rate, when conventional radiologic and ultrasound techniques fail.     

Primary pleural lymphoma: an unusual presentation of childhood non-Hodgkin lymphoma

A case of primary pleural non-Hodgkin lymphoma in a 2.5-year-old girl is reported. The patient had pleural involvement as the initial and only manifestation of the disease. Histopathalogic examination showed lymphoblastic lymphoma of T-cell origin. The child received the modified LSA2-L2 protocol. During the maintenance treatment, she had an isolated central nervous system relapse and died of neutropenic sepsis. To the authors' knowledge this represents the first case report of primary pleural lymphoma in the childhood period.     

Flow cytometric immunophenotyping in the diagnosis and follow-up of immunodeficient children

From time to time, paediatricians are confronted with children who might suffer from a primary immunodeficiency disease. For practical purposes, these children can be divided into four main clinical categories: (1) a relatively large group of children with recurrent ear-nose and throat and lower respiratory tract infections, in some cases caused by deficiencies of antibodies or complement; (2) children with failure to thrive, intractable diarrhoea or an opportunistic infection which can be caused by a T-lymphocyte or combined immunodeficiency; (3) children with infections with pyogenic bacteria or fungi as seen in case of granulocyte/monocyte function deficiency; and (4) a small heterogeneous group of children with recurrence of particular infections. Also, acquired immunodeficiency becomes a more common problem in paediatric practice. Flow cytometric immunophenotyping of leucocytes appears to be an efficient and rapid tool in the diagnosis and follow-up of immunodeficient patients, supporting early recognition, before serious infections have compromised the child's general condition. This technique can now be performed in many hospitals. In this review, we give directions for the use of flow cytometric immunophenotyping of leucocytes in the diagnosis and follow-up of immunodeficient children according to the four main clinical categories.     

小儿非霍奇金淋巴瘤的分型及治疗

目的 总结24例小儿非霍奇金淋巴瘤（NHL）的诊断及治疗。方法 采用组织病理及免疫组化诊断，并行临床分期，依据类型，分期选择治疗方案。结果 23/24例为晚期NHL，3例小无裂细胞型均为B细胞型，5例淋巴母细胞型均为T细胞型，大细胞型8例显示不均一的表现，治疗的完全缓解率为94.4%(17/18例）。10例长期治疗患儿目前无病存活7例。结论 依据病理，免疫类型及分期采用强化多药联合的大剂量化疗治疗小儿晚期NHL，可以达到较高的长期无病存活率。     

Flow cytometric immunophenotyping: principles and pitfalls

Within the last decade flow cytometry (FCM) has become an integral part of basic immunological research. Elaboration of this technology has been intensively stimulated by a rapidly growing sophistication in monoclonal antibody technology and vice versa. At present numerous applications are established that allow an increasingly detailed insight into the immune system, however, automation still must be considered the "cinderella of the arts". Thus, transition of this powerful approach from a basic to a routine clinical procedure is much more difficult than expected. Sufficient usage of flow cytometers still requires some knowledge of physics and its technical applications. Moreover, several problems arise from the complexity of the biological systems investigated. Here we give a brief introduction to immunofluorescence and FCM followed by a discussion of six exemplary pitfalls that we hope will emphasise the general importance of methodological considerations.     

Male gonadal function in survivors of childhood Hodgkin and non-Hodgkin lymphoma

The aim of this study was to investigate the impact of therapy on long-term gonadal function of young people cured of childhood lymphomas and to assess whether a prepubertal state during the treatment protects the gonads from chemotherapy and/or radiotherapy late effects. Clinical evaluation, semen analysis, and endocrine status were studied in 20 survivors of childhood lymphomas. Five patients received Inverted Y radiotherapy, 2320 cGy (1550-4000); all 20 received chemotherapy as follows: MOPP/ABVD protocol, 9 patients; COMP protocol, 5 patients; MOPP protocol, 3 patients; other protocols, 3 patients. Semen analysis results were as follows: normal values, 4/20 patients; oligospermia, 8/20 patients; azoospermia, 8/20 patients; FSH above normal level, 10/20 patients; 4/5 who received Inverted Y irradiation were azoospermic and 1 was severely oligospermic. Treatment damage to the testis involves tubular germinal elements. Radiotherapy and chemotherapy combinations that included nitrogen mustard or cyclophosphamide were associated with high rates of oligospermia and azoospermia. MOPP/ABVD combination did not have a significant better outcome of sperm counts compared to MOPP alone. Age at chemotherapy did not correlate with the sperm count; hence a prepubertal state did not protect the gonad from the late effects of treatment.     

Acute blindness as a presenting sign in childhood non-Hodgkin lymphoma

Non-Hodgkin lymphoma is the third most common malignancy in childhood. Occasionally, a patient with lymphoma may present with neuroophthalmologic symptoms and signs. It is usually associated with 3rd, 4th, and 6th cranial nerve palsies. Here, we report three children presenting with loss of vision. Two of them were cured with modified LMB-89 (lymphoma malignant B) chemotherapy regimen and radiotherapy. In one patient, loss of vision returned to normal in the left eye but persisted in the right eye. The second patient suffered from permanent visual loss, although the primary disease responded well to treatment. Unfortunately, the third patient died at an early phase of treatment. Acute blindness as a presenting symptom is rare in lymphoma. Early diagnosis and treatment is critical to increase the survival rate and to improve the quality of the vision.     

Long-term pulmonary function in survivors of childhood Hodgkin disease and non-Hodgkin lymphoma

BACKGROUND: The aim of our study was to evaluate the long-term effects of chemotherapy and/or radiotherapy on lung function in 75 childhood Hodgkin disease (HD) and non-Hodgkin lymphoma (NHL) survivors several years after treatment. PATIENTS AND METHODS: We studied 37 HD and 38 NHL survivors. These patients were divided into two groups according to the treatment protocols applied. Group I consisted of 23 patients who were treated with both chemotherapy and thoracic irradiation and Group II consisted of 52 patients who were treated with chemotherapy and no thoracic irradiation. A detailed history of smoking habits, respiratory symptoms, and diseases was recorded. Complete physical examinations and pulmonary function tests [PFT, including spirometry, lung volume, and diffusion capacity for carbon monoxide (DLCO)] were performed on all subjects. RESULTS: No patients reported acute or chronic respiratory symptoms or diseases. Pulmonary function abnormality (reduced lung volume and diffusion capacity) was found in 13% of patients at a median 5 years after diagnosis. The percentage of predicted normal value of forced expiratory volume in the 1st sec (FEV(1)), residual volume (RV), and DLCO were significantly lower in Group I than these values for Group II. There were no significant differences in PFT parameters between patients with HD and NHL (P > 0.05). It appears that the risk of reduced lung function was greater the younger the patient in therapy. CONCLUSION: Chemotherapy or chemo-radiotherapy-induced pulmonary sequalae in childhood may remain asymptomatic for many years.     

Primary adrenal insufficiency in childhood and adolescence: advances in diagnosis and management

OBJECTIVES: Primary adrenal insufficiency occurring in childhood and adolescence is due to abnormalities of gland development, gland responsiveness, and steroid biosynthesis or target organ response. Causes include autoimmune Addison's disease, tuberculosis, HIV, adrenoleukodystrophy, adrenal hypoplasia congenita and syndromes including triple A and IMAGe. We aimed to define the causes of adrenal insufficiency for a cohort of children in Melbourne. METHODS: We reviewed the frequency and variety of presentation of primary adrenal insufficiency to the Royal Children's Hospital over the past 10 years through an audit of patient records, collating demographic information, presentation and investigations. RESULTS: Sixteen cases (13 male, 3 female) of primary adrenal insufficiency were diagnosed at this hospital between January 1993 and July 2003. Median age at presentation was 7.7 years (range: birth to 14.8 years). Symptoms at presentation included weakness, increased pigmentation, abdominal pain, nausea, developmental delay or a reduction in school performance. Four patients presented with adrenal crisis. Median adrenocorticotrophic hormone (ACTH) at diagnosis was 246 pmol/L (range 30-969 pmol/L). Autoantibodies were positive in five patients. Five patients had elevation of very long chain fatty acids. Five patients were diagnosed with autoimmune adrenal insufficiency, five with adrenal hypoplasia congenita, five with adrenoleukodystrophy and one with IMAGe syndrome. CONCLUSIONS: A high index of suspicion results in earlier detection and possible prevention of adrenal crisis with a reduction in associated morbidities. Definitive diagnosis is now possible for almost all cases of primary adrenal insufficiency using technologies for screening autoimmunity, adrenoleukodystrophy (ALD) and genetic screening.     

Sonography in the diagnosis of Hodgkin's disease in childhood and adolescence

We are reporting about the results of the staging by ultrasound in 11 children with Hodgkin's disease. Sonography stands the test as a method that reliably predicts abdominal involvement in patients with Hodgkin lymphoma. Only in a few cases an indication for an additional computerized tomography is given. A demonstration of typical ultrasonic findings takes place.     

Early indicators of self-destruction in childhood and adolescence: a survey of pediatricians and psychiatrists

This paper reports a comparison of pediatricians' and psychiatrists' opinions about screening for children and adolescents at risk for self-destruction. Fifty-nine percent of the members of the Nebraska Chapter of the American Academy of Pediatrics and 69% of the members of the Nebraska District Branch of the American Psychiatric Association completed questionnaires containing selected early indicators for self-destruction and programs for suicide prevention. Principal findings were that pediatricians placed less emphasis than child psychiatrists on major depressive disorder, younger pediatricians placed more emphasis than psychiatrists on psychosocial indicators, physicians from both groups said they usually hospitalize attention-seeking self-destructive children, and pediatricians from smaller communities expressed less confidence in referral to psychiatrists but more confidence in community-based programs. Findings suggest that a small group of children and adolescents at risk for eventual suicide may not be identified as early as the more obvious larger groups of individuals who are accident prone, who ingest toxic substances, and who come from violent families. General indicators of risk for self-destruction include substance abuse, psychosocial problems, and minor depression. Factors indicating more specific risk for suicide include escalating stress, family enmeshment, and major mental illness, particularly major depressive disorder. Postgraduate education, instruments for discriminate screening, and balanced programs for suicide prevention are suggested to improve care.     

Systemic hemophagocytosis masking the diagnosis of large cell non-Hodgkin lymphoma

An 11-year-old female presented with clinical features suggestive of malignant histiocytosis: fever, weight loss, subcutaneous nodules, pulmonary infiltrates, adenopathy, and hepatosplenomegaly. On biopsy, lymph node and bone marrow demonstrated necrosis and extensive hemophagocytosis with no definitive evidence of malignancy; the subcutaneous nodules, however, demonstrated large-cell non-Hodgkin lymphoma. This clinicopathologic picture has been reported in adults, but not in children. Although serum G-CSF, M-CSF, and TNF levels were not elevated in this child, it is possible that other cytokines induced either directly or indirectly by the sub-cutaneous lymphoma resulted in hemophagocytosis. Med. Pediatr. Oncol. 29: 167–169, 1997. © 1997 Wiley-Liss, Inc.     

MDM2的表达与儿童非霍奇金淋巴瘤关系的研究

目的探讨儿童非霍奇金淋巴瘤(non-Hodgkin lymphoma, NHL)癌基因MDM2(murine double minute 2)的表达与NHL的关系.方法用免疫组化S-P法,检测NHL、对照组患儿病理组织MDM2蛋白的表达,用逆转录聚合酶链反应法(RT-PCR)检测NHL、对照组病理组织和NHL外周血单个核细胞MDM2 mRNA的表达.结果 (1)MDM2蛋白表达率为64.5%,MDM2 mRNA表达率为61.3%,与对照组比较均有显著意义(前者P<0.05,后者P<0.01).(2)MDM2蛋白表达率与NHL工作分类、细胞源分类、性别、临床分期、结外侵犯位点间差异没有显著性(P>0.05);与B状态、乳酸脱氢酶增高间差异有显著性(P<0.05).MDM2 mRNA表达率与工作分类、细胞源分类、性别、临床分期间差异无显著性(P>0.05);与B状态间差异有显著性(P<0.05);与结外侵犯部位、LDH增高间差异有非常显著性(P<0.01).(3)病理组织MDM2 mRNA与MDM2蛋白过表达率、外周血MDM2 mRNA过表达率间进行比较,结果存在关联性(P>0.05,kappa=0.655和0.571),病理组织MDM2蛋白过表达率与外周血MDM2 mRNA过表达率间差异也存在关联性 (P>0.05,kappa=0.609)).结论(1)儿童NHL患者MDM2基因的过度表达率较高.(2)NHL MDM2蛋白的过表达率与mRNA过表达率的结果基本一致.(3)MDM2过表达与儿童NHL患者的不良状况、不良预后有关.