Castleman disease: surgical cure in pediatric patients

Castleman disease is a rare disorder characterized by lymphoid hyperplasia which rarely manifests in children. We present 2 cases which highlight both histologic variants of this disease, and provide suggestions regarding workup and treatment with the goal of making practitioners aware of Castleman disease in the differential diagnosis of a child presenting with vague symptoms.     

aVR sign as a risk factor for life-threatening arrhythmic events in patients with Brugada syndrome

BACKGROUND: Risk stratification in Brugada syndrome (BS) is controversial, especially in asymptomatic individuals. OBJECTIVE: The aim of this study was to evaluate the significance of lead aVR in patients with BS. METHODS: Twenty-four patients with the electrocardiogram pattern of BS (24 male, mean age 32.1 +/- 13.6 years) and 24 healthy age- and gender-matched controls were studied. RESULTS: Thirteen patients were symptomatic. The R-wave amplitude or R/q ratio in lead aVR was significantly greater in patients experiencing a recurrence compared with those who did not. The aVR sign was defined as R wave >/= 0.3 mV or R/q >/= 0.75 in lead aVR. Most of the recurrences (78%) were in patients with present aVR sign; 84% of BS patients with present aVR sign had events during follow-up. In contrast, only 27% of BS patients with absent aVR sign had events during follow-up. CONCLUSION: Our study shows significant correlation between a prominent R wave in lead aVR (aVR sign) and risk for development of arrhythmic events in BS. In the presence of BS, prominent R wave in lead aVR may reflect more right ventricular conduction delay and subsequently more electrical heterogeneity, which in turn is responsible for a higher risk of arrhythmia.     

Veinous thrombectomy. Six case reports]

The aim of this study is to raise up the effect of surgical thrombectomy among other alternative therapies. This retrospective study reports 6 patients (mean age 63 years) admitted with phlegmasia cerulea dolens. All patients underwent surgical venous thrombectomy associated with infracava filter insertion in 2 cases. One patient died in the early postoperative course. In all other cases we noticed good early and late outcome both on clinical examination and duplex scanning assessment. In conclusion, surgical venous thrombectomy can be considered as a good and efficient procedure in the presence of phlegmasia cerulea dolens in order to relieve ischemia and to prevent whenever possible severe chronic venous disorders. However, fibrinolytic therapy might achieve as good results as surgery. Thus, the latter is to be reserved to very severe veinous ischemia with limb loss threatening where fibrinolytic therapy fails or is contre-indicated.     

Primary pulmonary mucosa-associated lymphoid tissue lymphoma with associated fungal ball in a patient with human immunodeficiency virus infection

We describe a case of primary mucosa-associated lymphoid tissue lymphoma of the lung in a 44-year-old man with human immunodeficiency virus. Low-grade pulmonary lymphomas in human immunodeficiency virus-positive patients are rare and are described most commonly in pediatric patients. The gross, histologic, and molecular features of this unusual case are described.     

Actinomyces and actinobacillus actinomycetemcomitans-Actinomyces-associated lymphadenopathy mimicking lymphoma

We present 2 unusual cases of long-standing, extensive reactive lymphadenopathy secondary to Actinomyces infection, 1 of which was also accompanied by Actinobacillus actinomycetemcomitans-Actinomyces complex infection. To our knowledge, histologic features of lymph node involvement by these organisms have not been previously reported in the literature. One patient had extensive cervical, posterior mediastinal, and abdominal lymphadenopathy. The second patient presented with a submandibular mass and cervical lymphadenopathy. Clinical features strongly suggested lymphoma. The histologic examination of the lymph nodes from both patients revealed reactive follicular hyperplasia, marked interfollicular and capsular fibrosis, and multiple interfollicular microabscesses. Characteristic Actinomyces colonies were identified at the center of the microabscesses in deep sections. Cultures were obtained from the lymph nodes of 1 patient, and were positive for A actinomycetemcomitans. Both patients had poor dental hygiene. Lymphadenopathy subsided with antibiotic therapy and appropriate dental care.     

Analysis of MHC genes in a Tunisian isolate with autoimmune thyroid diseases: implication of TNF -308 gene polymorphism

Autoimmune thyroid diseases (AITDs), which include Hashimoto thyroiditis (HT), Graves' disease (GD) and primary idiopathic myxoedema (PIM), are recognized as multifactorial diseases. In this study, we have examined single and haplotypic genetic variation across the major histocompatibility complex (MHC) in a Tunisian isolate with a high prevalence of AITDs (62 patients: 32 with GD, 9 with HT and 21 with PIM). Genotyping was performed for HLA class I and II alleles as well as polymorphisms within tumor necrosis factor (TNF), lymphotoxin alpha (TLalpha) and heat shock protein (HSP70-02 and HSP70-hom) genes. Our results showed association of HLA-A2-B50-TNF 2 haplotype with AITDs (p = 0.045). Linkage analysis using Simwalk2 program has shown significant result with TNF -308 gene polymorphism (p = 0.03). The FBAT has given evidence for genetic association with TNF -308 and HLA-DR gene polymorphisms. TNF 2 allele was associated with GD (p = 0.0011), whereas TNF 1, HLA-DR11 and DR12 (p = 0.0039, p = 0.00089 and p = 0.0056, respectively) were rather implicated in HT pathogenesis. Results found by TDT-STDT have confirmed the involvement of the TNF -308 gene polymorphism in AITD pathogenesis (p < 10(-9)).