Primary Cauda Equina T-Cell Lymphoblastic Lymphoma.

BACKGROUND: T-cell lymphoblastic lymphoma (T-LBL) is a rare and aggressive form of non-Hodgkin lymphoma. This report describes, to our knowledge, the first adult case of a primary cauda equina T-LBL. Treatment consists of multiagent chemotherapy, and surgical removal of T-LBL does not improve outcome. We discuss the workup of patients with an intradural spinal mass, together with a review of the literature on primary spinal lymphoma of the cauda equina. CASE DESCRIPTION: A 54-year-old woman with Crohn's disease, for which she was taking immunosuppressive medication, presented with progressive back pain radiating to both legs and deteriorating neurologic deficits caused by an intradural, contrast-enhancing lesion in the L1-5 region. During acute surgery, the tumor was partially resected. Immunohistochemical phenotyping revealed a T-LBL. No other lymphoma localizations were found after subsequent staging. Despite extensive treatment, the patient died of disseminated disease throughout the central nervous system, 6 weeks after the diagnosis. CONCLUSIONS: Pain and progressive neurologic complaints can be symptoms of a (malignant) intradural spinal tumor. Intradural lymphoma must be considered as a differential diagnosis by clinicians because it can mimic neoplasms that often require urgent surgery. The histopathologic diagnosis should preferably be obtained by way of cerebrospinal fluid analysis or tumor biopsy because tumor resection has no beneficial effect on the oncologic outcome.

Vitamin A and beta (ß)-carotene supplementation for cystic fibrosis.

BACKGROUND: People with cystic fibrosis (CF) and pancreatic insufficiency are at risk of a deficiency in fat-soluble vitamins, including vitamin A. Vitamin A deficiency predominantly causes eye and skin problems, while excessive levels of vitamin A can harm the respiratory and skeletal systems in children and interfere with the metabolism of other fat-soluble vitamins. Most CF centres administer vitamin A as supplements to reduce the frequency of vitamin A deficiency in people with CF and to improve clinical outcomes such as growth, although the recommended dose varies between different guidelines. Thus, a systematic review on vitamin A and vitamin A-like supplementation (carotenes or other retinoids) in people with CF would help guide clinical practice. This is an update of an earlier Cochrane Review. OBJECTIVES: To determine if supplementation with vitamin A, carotenes or other retinoid supplements in children and adults with CF reduces the frequency of vitamin A deficiency disorders, improves general and respiratory health and affects the frequency of vitamin A toxicity. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Cystic Fibrosis Trials Register compiled from electronic database searches and handsearching of journals and conference abstract books. Additionally we searched several ongoing trials registries, including ClinicalTrials.gov, the WHO International Clinical Trials Registry Platform and the International Standard Randomised Controlled Trial Number Registry.Most recent database searches: 01 June 2018. SELECTION CRITERIA: All randomised or quasi-randomised controlled studies comparing all preparations of oral vitamin A, carotenes or retinoids (or in combination), used as a supplement compared to placebo at any dose, for at least three months, in people with CF (diagnosed by sweat tests or genetic testing) with and without pancreatic insufficiency. DATA COLLECTION AND ANALYSIS: Two authors individually assessed study quality and extracted data on outcome measures. The authors assessed the quality of the evidence using the GRADE system. Investigators were contacted to retrieve missing quantitative data. MAIN RESULTS: No studies of vitamin A or other retinoid supplementation were eligible for inclusion. However, one randomised study of beta (ß)-carotene supplementation involving 24 people with CF who were receiving pancreatic enzyme substitution was included. The study compared successive ß-carotene supplementation periods (high dose followed by low dose) compared to placebo. The results for the low-dose supplementation period should be interpreted with caution, due to the lack of a wash-out period after the high-dose supplementation.The included study did not report on two of the review's primary outcomes (vitamin A deficiency disorders and mortality); results for our third primary outcome of growth and nutritional status (reported as z score for height) showed no difference between supplementation and placebo, mean difference (MD) -0.23 (95% confidence interval (CI) -0.89 to 0.43) (low-quality evidence). With regards to secondary outcomes, supplementation with high-dose ß-carotene for three months led to significantly fewer days of systemic antibiotics required to treat pulmonary exacerbations, compared to controls, MD -15 days (95% CI -27.60 to -2.40); however, this was not maintained in the second three-month section of the study when the level of ß-carotene supplementation was reduced, MD -8 days (95% CI -18.80 to 2.80) (low-quality evidence). There were no statistically significant effects between groups in lung function (low-quality evidence) and no adverse events were observed (low-quality evidence). Supplementation affected levels of ß-carotene in plasma, but not vitamin A levels. The study did not report on quality of life or toxicity. AUTHORS' CONCLUSIONS: Since no randomised or quasi-randomised controlled studies on retinoid supplementation were identified, no conclusion on the supplementation of vitamin A in people with CF can be drawn. Additionally, due to methodological limitations in the included study, also reflected in the low-quality evidence judged following the specific evidence grading system (GRADE), no clear conclusions on ß-carotene supplementation can be drawn. Until further data are available, country- or region-specific guidelines regarding these practices should be followed.

Comparison of bronchoscopy and bronchoalveolar lavage findings in three types of suppurative lung disease.

BACKGROUND: Endobronchial suppuration is present in children with protracted bacterial bronchitis (PBB), bronchiectasis, and cystic fibrosis (CF). However, no studies have directly compared bronchoscopy and bronchoalveolar lavage (BAL) findings across these conditions within a single center using the same techniques and with shared community pathogens. AIM: To determine; (i) the bronchoscopic findings and BAL microbiology and cellularity among children with these conditions and; (ii) the relationship between bacterial pathogens, airway cellularity and aberrant macroscopic bronchoscopic findings. METHODS: We retrospectively reviewed all bronchoscopy data (undertaken over 6.5-years) from our center in children (<6 years; n = 316) meeting definitions of PBB (n = 125), bronchiectasis (n = 138), and CF (n = 53). RESULTS: The children's median age was 26-months (Interquartile range (IQR) = 16-43). Children with PBB and bronchiectasis had higher rates of Haemophilus influenzae, Moraxella catarrhalis, and Streptococcus pneumoniae infection, whereas children with CF had frequent Pseudomonas aeruginosa and Staphylococcus aureus infections. Novel findings include detection of cytomegalovirus and Epstein-Barr virus (EBV) (by polymerase chain reaction) in children with PBB (26%, 17%, respectively) and bronchiectasis (27%, 29%). Median airway neutrophil percentage was significantly higher in CF (68%; IQR = 42-83) compared to PBB (36%; IQR = 18-68) and bronchiectasis (22%; IQR = 8-64) (P < 0.0001), despite lower rates of infection. Presence of malacia did not significantly impact on infection or inflammation. CONCLUSION: In this first study to directly compare bronchoscopic data among young children with PBB, bronchiectasis, and CF, microbiological patterns of airway infections and neutrophilia varied. Our findings of cytomegalovirus and EBV detection in children with PBB and bronchiectasis require confirmation and further evaluation.