Pulmonary alveolar proteinosis coexisting with homozygous hemoglobin E

Publish : December 15, 2012
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Niramol Chanplakorn1, Atcharaporn Pongtippan1, Harutai Kamalaporn2
1Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
2Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand


Background: Pulmonary alveolar proteinosis (PAP) is an uncommon lung disorder of obscure and possibly diverse causes characterized by abnormal accumulation of lipoproteinaceous surfactant material in alveoli. The prevalence of acquired PAP has been estimated to be 0.37 per 100,000 populations. We present a case of PAP coexisting in homozygous hemoglobin (Hb) E.

Case presentation: A 13-year-old girl who had been diagnosed as homozygous Hb E. She presented with dyspnea and cough. Thoracotomy was performed due to abnormal mediastinal mass disclosed in CT scan. However, only slightly thymus gland enlargement was found then wedge lung biopsy was performed. Examination of the sections of lung revealed alveoli diffusely fi lled with eosinophilic granular material. Bron- choalveolar lavage was also performed. Cytopathologic evaluation revealed a milky appearance with micro- scopically amorphous granular material with positive periodic acid-Schiff stain and scant chronic infl amma- tory cells. Findings were consistent with PAP. The clinical condition of the patient was stable after treatment.


Address of editorial Corresponce
✎  Vorachai Sirikulchayanonta, MD, Editor-in-Chief: Asian Archives of Pathology, Faculty of Science, Rangsit University, Pathumthani 12000, Thailand.
✉  Email address: asianarchpath@gmail.com, vorachai7@gmail.com