Acute promyelocytic leukemia

Introduction: The patterns of disease in invasive pulmonary aspergillosis are protean. An unusual variant of this disease, pseudomembranous tracheobronchial aspergillosis was first reported by Pervex et al.[1] We describe a case of suspected graft-versus-host disease of the lung that evolved rapidly to respiratory failure and death from this entity.

Case Presentation: A 20 year old Chinese student was diagnosed to have acute myeloid leukaemia in August 96 when he presented with exertional dyspnea secondary to anaemia (hemoglobin 7.3 g/dL). After undergoing chemotherapy (a regime involving cyclophosphamide) and total body irradiation, he underwent an allogeneic bone marrow transplant in November 96. About 2 years later in July 98, he developed exertional dyspnoea again, despite a normal hemoglobin of 14.0 g/dL. Spirometry revealed severe airway obstruction with an [FEV.sub.1] (forced expiratory volume in the first second) of 1.66 L (37.2% predicted). His pretransplant spirometry had been normal ([FEV.sub.1] 4.44 L in October 96). High resolution Computed Tomography of the chest revealed hyperinflated lungs with bilateral perihilar bronchial thickening consistent with chronic obstructive lung disease. He was treated with bronchodilators and his immunosuppressive agents were adjusted for possible graft versus host disease of the lung. He was admitted in November 98 for an episode of spontaneous pneumomediastinum. This resolved with conservative management, but he subsequently began to develop chronic diarrhoea. He was admitted from the outpatient clinic in December 98 for the problem of possible graft-versus-host disease of the gastrointestinal tract. Seven days after admission, he developed acute dyspnea and respiratory failure. The chest roentgenogram showed recurrence of the pneumomediastinum. He required emergent bilateral chest tube thoracostomies, endotracheal intubation and mechanical ventilation. Bronchoscopy done 3 days later showed an extensive whitish pseudomembrane in the tracheobronchial tree. Bronchial biopsy and bronchoalveolar lavage confirmed the diagnosis of pseudomembranous tracheobronchial aspergillosis. The patient died from progressive respiratory failure 6 days after endotracheal intubation despite therapy intravenous and nebulized amphotericin B.

Discussion: Although chronic graft-versus-host disease of the lung may explain the airway obstruction at presentation in this case, early aspergillous tracheobronchitis is a diagnostic differential. Bronchoscopy was diagnostic of the pseudomembranous aspergillus tracheobronchitis that the patient developed late in the course of the infection.

Conclusion: The overall outcome of immunocompromised patients with invasive pulmonary aspergillosis remains poor, as the diagnosis is frequently made late. Physicians should be aware of the spectrum of pulmonary aspergillosis including this rapidly evolving entity, as earlier recognition and diagnosis is essential to improve treatment response.[2]

References

[1] Pervex NK, Kleinerman J, Kattan M, et al. Pseudomembranous necrotizing bronchial aspergillosis: A variant of invasive aspergillosis in patients with hemophilia and acquired immune deficiency syndrome. Am Rev Respir Dis 1985; 131:961-963

[2] Pursell KJ, Telzak EE, Armstrong D. Aspergillus species colonization and invasive disease in patients with AIDS. Clin Infect Dis 1992; 14:141

Kay-Leong Khoo, V. S. Doshi, Philip Eng--Department of Respiratory & Critical Care Medicine, Singapore General Hospital, Singapore

COPYRIGHT 1999 American College of Chest Physicians
COPYRIGHT 2000 Gale Group