A breathless female

Abstract

A 67-yr-old female was admitted with a two week history of dry cough, cold sores and progressive breathlessness. Her exercise tolerance had gradually reduced over 3 yrs from 3 miles to half a mile. Three years previously she was diagnosed as having chronic myeloid leukaemia for which she received a total of 1.3 g busulphan, producing remission for the last 2 yrs. She was an exsmoker with a 40 pack-yr history. There was no relevant occupational history. The only regular medication was allopurinol.On examination she was breathless at rest, cyanosed but afebrile, pulse rate 110 beats . min -1 , respiratory rate 24 beats . min -1 , blood pressure 120/70, normal jugular venous pressure and heart sounds. On examination of the chest, percussion notes were normal and auscultation revealed bilateral basal crackles. The remainder of the examination was normal.Full blood count showed normocytic normochromic anaemia with haemoglobin (Hb) 88 g . L -1 and leukocytosis with white blood cell (WBC) count 17.2610 9 cells . L -1 , erythrocyte sedimentation rate (ESR) was raised to 80 (normal 0±20) and C-reative protein (CRP) was raised to 164 mg . L -1 (normal range 0±9). The chest radiograph on admission is shown in figure 1. Autoimmune profile, blood culture, sputum culture, urine culture, viral serology and protein electrophoresis were normal. Arterial blood gas analysis on air showed arterial oxygen tension (Pa,O 2 ) 3.5 kPa and arterial carbon dioxide tension (Pa,CO 2 ) 3.3 kPa, the Pa,O 2 increasing to 11.7 kPa on 50% oxygen. Dynamic spirometry showed a restrictive ventilatory defect forced expiratory volume in one second (FEV1) 0.95 (61% pred), forced vital capacity (FVC) 0.97 (51% pred), FEV1/FVC ratio 98%. Shunt fraction determined by breathing 100% oxygen was grossly elevated at 40%.High-resolution computed tomography (HRCT) was performed ( fig. 2). Fibreoptic bronchoscopy showed normal endobronchial anatomy. Owing to severe hypoxia, bronchoalveolar lavage (BAL) was not performed. Fluid obtained from bronchial washing was colourless and was negativeforPneumocystiscariniipneumonia(PCP).Transbronchial biopsy concluded at the same time confirmed the diagnosis ( fig. 3).

Proteinosis alveolar, un hallazgo incidental en un estudio preoperatorio con respuesta insuficiente al lavado pulmonar completo

“…Para ello, además de la historia clínica, exploración física y datos analíticos, la TAC-AR y el estudio del material obtenido en el lavado broncoalveolar (BAL) que habitualmente presenta un aspecto lechoso, constituido fundamentalmente por fosfolípidos, sobre todo lecitina, además de otras proteínas (1,4,8,12,14). La biopsia transbronquial puede ser diagnóstica en ocasiones, aunque dada la afectación parcheada característica de esta enfermedad una biopsia que muestra parénquima pulmonar normal no es excluyente.…”