Lymphomatoid granulomatosis (LG) is a rare T cell rich, B cell non-Hodgkin's lymphoma which is difficult to diagnose. We present a patient with LG who demonstrated many of the difficulties in diagnosis and highlighted the importance of reviewing the diagnosis if treatment does not have the anticipated effect.A 49 year old male smoker presented to another hospital with a 3 month history of weight loss and sweats. A chest radiograph showed a mass in the left lower lobe and bronchoscopic examination revealed inflammation involving the left lower lobe bronchus. Washings were negative for organisms and malignancy. A computed tomographic (CT) scan showed a cavitating mass in the left lower lobe with multiple smaller opacities throughout both lung fields. Percutaneous fine needle aspiration of the mass showed inflammation with necrotic debris. He was discharged but presented 1 week later with fever and rigors and was transferred to this hospital.On arrival he had a cough productive of copious, foul smelling, purulent sputum. His temperature was 40˚C. There was no lymphadenopathy or hepatosplenomegaly. Neurological examination was normal. C-reactive protein (CRP) was 59 mg/l. Blood, urine and sputum cultures, white cell count, serum angiotensin converting enzyme, P-and C-ANCA, autoantibody screen, complement, test for HIV, viral screening, and brucella titres were all negative or normal. A repeat bronchoscopic examination was non-diagnostic. A repeat CT scan showed a 7 cm cavitating lesion in the left lower lobe with an air/fluid level and minor ''inflammatory changes'' in the right lung.Empirical treatment for a suspected lung abscess was commenced using intravenous benzylpenicillin, gentamicin, and metronidazole. Over the next 21 days his pyrexia settled, sputum cleared, and the CRP level normalised. The chest radiograph remained unchanged. Four weeks later his purulent sputum, fever, and raised CRP level recurred. Open lung biopsy was considered, but the thoracic surgical team declined because of the evidence for active infection. Antibacterial therapy was recommenced with intravenous cefotaxime and metronidazole. As before, there was a good clinical response but without radiological resolution. Further symptomatic relapse occurred once the drugs were withdrawn. Seven months after initial presentation he deteriorated further. A CT scan showed the left lower lobe cavity unchanged but there were now numerous nodular densities throughout the right lung (fig 1). Repeat bronchial biopsies showed abnormal lymphoid tissue characterised by a polymorphous lymphoid infiltrate containing scattered enlarged blast-like cells. These stained positively for CD20, a B cell marker, and immunoglobulin rearrangement studies showed clonality. These findings suggested lymphomatoid granulomatosis.He was treated with methylprednisolone, vinblastine, and cyclophosphamide. Within 2 weeks his dyspnoea, fever, and right sided radiological abnormalities resolved. The left lower lobe lesion reduced by 40% after three cycles of chemotherapy. Howev...

Background: The majority of patients with cystic fibrosis (CF) are diagnosed in the first decade of life. In a small number of patients, the diagnosis is not made until later. Objective: In this study, the clinical and genetic features of patients diagnosed after the age of 10 were examined. Methods: All living patients in Northern Ireland diagnosed prior to 1983, when neonatal screening was introduced, were studied. A total of 103 patients were identified of whom 18 were diagnosed after the age of 10. The relationships between late diagnosis and clinical presentation, sputum microbiology, pancreatic sufficiency, nutritional status, genotype and distance from the regional CF centres was determined by multiple regression analysis. Results: All 18 late-diagnosed patients had a sweat (chloride >70 mmol/l). Late diagnosis was significantly related to carriage of the R117H mutation (r² = 0.45) and pancreatic sufficiency (r² = 0.37). There was a weak relationship with pulmonary function (r² = 0.09). Conclusions: In Northern Ireland, late diagnosis in mainly associated with pancreatic function and carriage of the R117H mutation.

No abstract

Acquisition of B. cepacia may be a cause of, rather than a marker for, a decrease in pulmonary function.

In clinically stable young adult patients with moderately severe respiratory disease total energy expenditure is comparable to that an a control population despite in increase in resting energy expenditure and both 24-h HR and doubly isotopically labelled water are suitable for use in patients with cystic fibrosis.