Author of

• 12853

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  P3.24 - Poster Session 3 - Supportive Care (ID 160)

  • Event: WCLC 2013
  • Type: Poster Session
  • Track: Supportive Care
  • Presentations: 1
  • Moderators:
  • Coordinates: 10/30/2013, 09:30 - 16:30, Exhibit Hall, Ground Level

  • 12868

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    P3.24-015 - Pulmonary adenocarcinoma in a patient with Hermansky-Pudlak Syndrome (ID 1071)

    09:30 - 09:30  |  Author(s): C. Simsek
    • Abstract

    Background
    Hermansky-Pudlak Syndrome (HPS) is a rarely seen autosomal recessive disease characterized by oculocutaneous albinism, bleeding tendency from platelet storage pool deficiency, and lysosomal occumulation of ceroid in the reticuloendothelial system. Ceroid occumulation in alveolary macrophages causes pulmonary inflammation and interstitial pneumonia. Pulmonary fibrosis is the most serious complication and main reason of mortality. Pulmonary fibrosis is also a well known etiology for the onset of lung carcinoma.

    Methods
    N/A

    Results
    A 64-year-old albino patient admitted with dyspnea, back pain, and weakness. He had a smoking history of 40 pack-years. In his past medical history, he has recurrent nasal bleeding, and he was given inhaler bronchodilators for dyspnea on exertion last year. Rutin laboratory analysis was normal. There was a mild hypoxemia (PaO2: 63 mmHg). There was a restirictive pulmonary functional deficit with a reduced diffusion capacity (%56). A thorax CT demonstrated pulmonary fibrosis, left pleural effusion, and irregularly marginated collapse and consolidation areas adjacent to pleural effusion. On PET/CT a mass lesion 6 cm in largest diameter (SUVmax: 15.6) is distiguised in left lower lobe superior segment. There was also a nodular pleural thickening (SUVmax: 13.5) on the left hemitorax and multiple mediastinal lymph nodes with high SUVmax levels. The presence of albinism, the history of bleeding diathesis, and the presence of pulmonary fibrosis suggested the diagnosis of HPS. The patient referred to ophthalmology consultation. There were hypopigmentation on coroid layer and iris. Hematology clinic comfirmed the presence of platelet dysfunction. After all the patient was diagnosed as HPS. After transfusion of platelets, a transtoracic needle biopsy was performed and the pathology was compatible with adenocarcinoma (Stage 4, pleural involvement). The patient refused any therapy and discharged from hospital with home oxygen therapy.

    Conclusion
    Herein we present a rare case with pulmonary fibrosis diagnosed simultaneously as HPS and pulmonary adenocarcinoma.