The development of pulmonary adenocarcinoma in a patient with Job's syndrome, a rare immunodeficiency condition

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...

Full description

Saved in:
Bibliographic Details
Published in:Tumori 2004-01, Vol.90 (1), p.132-135
Main Authors: Oztop, Ilhan, Demirkan, Binnaz, Tarhan, Oktay, Kayahan, Hasan, Yilmaz, Ugur, Kargi, Aydanur, Alakavuklar, Mehmet
Format: Article
Language:English
Subjects:
Adenocarcinoma - complications
Adenocarcinoma - diagnosis
Adenocarcinoma - immunology
Adenocarcinoma - secondary
Adult
Bone Neoplasms - secondary
Fatal Outcome
Humans
Job Syndrome - complications
Liver Neoplasms - secondary
Lung Diseases, Fungal - etiology
Lung Neoplasms - complications
Lung Neoplasms - diagnosis
Lung Neoplasms - immunology
Lung Neoplasms - pathology
Male
Male Urogenital Diseases - microbiology
Pseudomonas Infections - etiology
Sepsis - etiology
Sepsis - microbiology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0300-8916
2038-2529
DOI:10.1177/030089160409000126