title: The development of pulmonary adenocarcinoma in a patient with Job's syndrome, a rare immunodeficiency condition.
abstract: The hyperimmunoglobulin E (HIE) (Job's) syndrome often has it onset in childhood and is characterized by markedly elevated serum IgE levels, chronic dermatitis and recurrent pyogenic infections. Lymphoid malignancies have most commonly been associated with this syndrome while the first case in the literature of carcinoma associated with HIE syndrome was a squamous cell carcinoma of the vulva, described by Clark et al. in 1998. We observed a male patient with Job's syndrome diagnosed at age three who presented with bone pain and a metastatic epithelial tumor of the bone revealed by biopsy. Diagnostic procedures aimed at detecting the primary site showed multiple mediastinal lymph nodes with lung and liver metastases on computed tomography scans and an extradural spinal metastasis at the upper thoracic level on magnetic resonance imaging. Although the patient refused a bronchoscopic procedure, a diagnosis of pulmonary adenocarcinoma was established on the basis of sputum cytology and the clinical aspects of tumor extent. Intravenous corticosteroids and palliative radiotherapy were given for the spinal metastasis. Palliative chemotherapy could not be started because of the patient's poor performance status as well as nosocomial fungal pneumonia and pseudomonal urogenital infection with bacteremia. Despite the antifungal and broad-spectrum antimicrobial treatments, the patient died of pseudomonal sepsis.
authors.affiliation: Division of Hematology-Oncology, Dokuz Eylul University School of Medicine, Izmir, Turkey.
authors.forename: Ilhan
authors.initials: I
authors.lastname: Oztop
authors.forename: Binnaz
authors.initials: B
authors.lastname: Demirkan
authors.forename: Oktay
authors.initials: O
authors.lastname: Tarhan
authors.forename: Hasan
authors.initials: H
authors.lastname: Kayahan
authors.forename: Ugur
authors.initials: U
authors.lastname: Yilmaz
authors.forename: Aydanur
authors.initials: A
authors.lastname: Kargi
authors.forename: Mehmet
authors.initials: M
authors.lastname: Alakavuklar
mesh_terms: D000230:Adenocarcinoma / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000556:secondary; D000328:Adult; D001859:Bone Neoplasms / Q000556:secondary; D017809:Fatal Outcome; D006801:Humans; D007589:Job Syndrome / Q000150:complications*; D008113:Liver Neoplasms / Q000556:secondary; D008172:Lung Diseases, Fungal / Q000209:etiology; D008175:Lung Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D008297:Male; D052801:Male Urogenital Diseases / Q000382:microbiology; D011552:Pseudomonas Infections / Q000209:etiology; D018805:Sepsis / Q000209:etiology* / Q000382:microbiology