Respiratory medicine case reports
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Respir Med Case Rep · Jan 2015
Case ReportsDramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy.
Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH.
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Kaposi's sarcoma (KS) is a highly vascular tumour, which was first described by the Hungarian dermatologist Moritz Kaposi Kohn before the discovery of the human immunodeficiency virus (HIV). Historically, KS has been linked to immunosuppression or to elderly male patients, especially in relation to diffuse cutaneous KS. We describe a case of Bronchopulmonary Kaposi's sarcoma in a patient with AIDS who was successfully treated with HAART and Liposomal Doxorubicin chemotherapy.
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Respir Med Case Rep · Jan 2015
Case ReportsLung adenocarcinoma presenting with isolated 'chronic cough' of 3 years duration-a cautionary tale.
Chronic cough that is dry, non-productive and without constitutional symptoms is often thought to have a non-malignant etiology such as asthma, post-nasal drip or gastroesophageal reflux disease (GERD). We present a case of a patient with a 3 year history of 'chronic cough' that was dry, non-productive cough and without any constitutional symptoms. Initial chest x-ray (CXR) done 3 years ago showed some streaky atelectasis in the right middle lobe along with some volume loss on that side. ⋯ A PET scan subsequently confirmed a hypermetabolic right hilar mass causing extrinsic compression of the bronchus intermedius. She ultimately required a complete right pneumonectomy with partial pericardiectomy and had complete resolution of her cough. This case highlights the fact that 'chronic cough' should always be thoroughly investigated and should remain a diagnosis of exclusion until all sinister pathologies have been ruled out.
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Pulmonary tumour thrombotic microangiopathy (PTTM) is a rapidly progressive pulmonary disease that is a fatal complication of malignancy. It manifests clinically as subacute respiratory failure with pulmonary hypertension, progressive right sided heart failure, and sudden death. We describe here a case of PTTM associated with occult metastatic signet ring cell carcinoma of the stomach. Although rare, PTTM needs to be considered in the differential diagnosis of dyspnoea of unknown origin, particularly in patients with respiratory failure and also pulmonary hypertension, and in patients were there is no improvement in respiratory symptoms with steroid therapy.
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Respir Med Case Rep · Jan 2015
Case ReportsCommon variable immunodeficiency diagnosed during the treatment of bronchial asthma: Unusual cause of wheezing.
Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency in adults and children. We herein report a case of CVID, who was misdiagnosed with asthma due to wheezing episodes and relatively late onset. A 51-year-old woman had suffered from recurrent upper and lower airway infection for recent 2 years. ⋯ She was diagnosed with CVID, and was treated with replacement of gammaglobulin. Thereafter, her wheezing episodes with infection were remarkably improved. Because the delay of diagnosis with CVID likely causes poor mortality and morbidity, a possibility of CVID should be considered in patients with frequent asthma-like symptoms due to recurrent airway infection.