We report the case of a 51-year-old non-smoking man who presented with flu-like symptoms and patchy, migratory infiltrates as evidenced on a chest-X ray. No response to antibiotic therapy was noted. Transbronchial lung biopsy revealed BOOP-like reaction associated with histopathology characteristic of pulmonary histiocytosis X. Immunoperoxidase stain showed histiocytic cells being positive for S-100 protein and CD1a. Along with the Langerhans cell proliferation, a bronchiolitis obliterans organizing pneumonia-like reaction was noted. In this paper, we discuss in this patient medical course and the clinical and pathologic characteristics of this unusual presentation of pulmonary histiocytosis X. ABBREVIATIONS INTRODUCTION Pulmonary Histiocytosis X (PHX) is a rare granulomatous disease of unknown etiology characterized by ill defined granulomas containing a large number of Langerhans cells (LC). 1 It represents less than 4% of all forms of interstitial lung disease. The pathogenesis of PHX is related to an uncontrolled immune reaction initiated by LC, and clearly associated with cigarette smoking. PHX responds modestly to corticosteroid, and other anti-inflammatory agents. CASE REPORT A 51-year-old White man was admitted to the hospital after 2 weeks of dry cough, low-grade fever, generalized malaise, and nasal congestion. He was treated for a respiratory tract infection with 1500-mg Penicillin qd for 7 days, with minimal response. Clarithromycin was then added at 500 mg twice a day for 7 days; without significant improvement.The patient had a longstanding history of allergic rhinitis but no drug allergies. There were no environmental or occupational exposures. He did not smoke or abuse alcohol. He worked as a salesman.Physical examination showed a patient was not in distress and was alert and oriented. Vital signs were temperature, 38.5°C; respiratory rate was 20 breaths/min; blood pressure, 130/70; heart rate, 90; oxygen saturation, 96% on room air. Auscultation of the lungs revealed a bilateral ronchi and basilar crackles. The remaining physical examination was normal. The hemoglobin and white cell count was normal with mild eosinophilia. The pulmonary function tests showed a mild decrease in diffusing lung capacity.Chest-X radiograph (CXR) showed patchy infiltrates and consolidation in the left lower lobe (Fig 1.A). Lymphocytosis and eosinophilia were found in bronchoalveolar lavage. No organisms were identified.On admission to the hospital, intravenous antibiotics were started, and of respiratory symptoms improved. The CXR done at discharge showed partial resolution of the previous patchy infiltrates. After seven days the patient returned complaining of fever, chills, and malaise and the CXR showed new infiltrates on the right lower lobe (Fig 1.B). He underwent bronchoscopy and transbronchial lung biopsy revealed in light microscopic examination ill-defined granulomas containing an interstitial infiltrate with large number of histiocytes. The histiocytes showed reniform nuclei with longitudinal nuclear grooves and abundant pale, eosinophilic cytoplasm, which were consistent with LC (Fig 2.A). Histopathology was confirmed by immunoperoxidase staining positive for S-100 protein, and CD1a, (Fig 2.B), which confirm the diagnosis of PHX. 1 4 actually known as Langerhans cell granulomatosis. 2 In addition, granulation-tissue type fibrosis was observed obstructing the bronchioles and within alveolar spaces, which is compatible with bronchiolitis obliterans organizing pneumonia. (BOOP)(Fig 2.C)Corticosteroids were started and marked clinical and radiological improvement was noted in only one week (Fig 1.C) Chest-X radiograph (CXR) showed patchy infiltrates and consolidation in the left lower lobe (Fig 1.A).
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