The Development of Pulmonary Adenocarcinoma in a Patient with Job's Syndrome, A Rare Immunodeficiency Condition

Öztop, İlhan; Demirkan, Binnaz; Tarhan, Oktay; Kayahan, Hasan; Yılmaz, Uğur; Kargı, Aydanur; Alakavuklar, Mehmet

doi:10.1177/030089160409000126

Cited by 26 publications

(18 citation statements)

References 10 publications

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“…Complications such as intracranial and coronary arterial aneurysms [5][6][7][8] and malignancy including lymphoma [9][10][11] have been reported previously. AD-HIES results from mutations in the Signal Transducer and Activator of Transcription 3 (STAT3) transcription factor, which leads to a loss of STAT3 function, poor Th17 cell differentiation, aberrant CD8+ T cell differentiation, and abnormal B cell function [12][13][14][15][16][17][18][19][20][21].…”

Section: Introductionmentioning

confidence: 94%

Successful Haploidentical Donor Hematopoietic Stem Cell Transplant and Restoration of STAT3 Function in an Adolescent with Autosomal Dominant Hyper-IgE Syndrome

et al. 2015

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Section: Introductionmentioning

confidence: 94%

Successful Haploidentical Donor Hematopoietic Stem Cell Transplant and Restoration of STAT3 Function in an Adolescent with Autosomal Dominant Hyper-IgE Syndrome

et al. 2015

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“…Regarding the solid lung tumors , there are only few case reports in the literature ( leiomyoma, pulmonary adenocarcinoma ) and this respiratory complication is considered to be in general rare in PID patients (72, 73). Pulmonary comprise due to metastasis (origin from, e.g., gastric carcinoma) is more often seen than primary pulmonary malignancy (7).…”

Section: Respiratory Manifestations Of Primary Immunodeficienciesmentioning

confidence: 99%

Pulmonary Manifestations of Primary Immunodeficiency Disorders in Children

et al. 2014

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Primary immunodeficiencies (PIDs) are inherited disorders in which one or several components of immune system are decreased, missing, or of non-appropriate function. These diseases affect the development, function, or morphology of the immune system. The group of PID comprises more than 200 different disorders and syndromes and the number of newly recognized and revealed deficiencies is still increasing. Their clinical presentation and complications depend on the type of defects and there is a great variability in the relationship between genotypes and phenotypes. A variation of clinical presentation across various age categories is also presented and children could widely differ from adult patients with PID. Respiratory symptoms and complications present a significant cause of morbidity and also mortality among patients suffering from different forms of PIDs and they are observed both in children and adults. They can affect primarily either upper airways (e.g., sinusitis and otitis media) or lower respiratory tract [e.g., pneumonia, bronchitis, bronchiectasis, and interstitial lung diseases (ILDs)]. The complications from lower respiratory tract are usually considered to be more important and also more specific for PIDs and they determinate patients’ prognosis. The spectrum of the causal pathogens usually demonstrates typical pattern characteristic for each PID category. The respiratory signs of PIDs can be divided into infectious (upper and lower respiratory tract infections and complications) and non-infectious (ILDs, bronchial abnormalities – especially bronchiectasis, malignancies, and benign lymphoproliferation). Early diagnosis and appropriate therapy can prevent or at least slow down the development and course of respiratory complications of PIDs.

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“…Further studies have shown that patients with HIES do not seem to be at increased risk of other cancers. 21,22 …”

Section: Differential Diagnosismentioning

confidence: 99%

Hemoptysis in a Patient with Elevated Immunoglobulin E

Gernez

Tsuang

Smith

et al. 2016

The Journal of Allergy and Clinical Immunology: In Practice

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Recurrent pneumonia with cavitation leading to pneumatoceles, secondary fungal infections, and hemoptysis are major causes of mortality and morbidity in patients with hyper-IgE syndrome. Prevention and aggressive treatment of pneumonia in these patients are essential to prevent further lung damage, but treatment may be delayed because the classic signs/symptoms of infection such as fever, chills, or rigors may be lacking. Early imaging to identify infection is essential for diagnosis and treatment. The mainstay of therapy is continuous, full-dose daily trimethoprim-sulfamethoxazole and commonly fungal coverage. Because hyper-IgE syndrome is a progressive disease, patients’ condition may worsen despite compliance with prophylactic therapy.

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The Development of Pulmonary Adenocarcinoma in a Patient with Job's Syndrome, A Rare Immunodeficiency Condition

Cited by 26 publications

References 10 publications

Successful Haploidentical Donor Hematopoietic Stem Cell Transplant and Restoration of STAT3 Function in an Adolescent with Autosomal Dominant Hyper-IgE Syndrome

Successful Haploidentical Donor Hematopoietic Stem Cell Transplant and Restoration of STAT3 Function in an Adolescent with Autosomal Dominant Hyper-IgE Syndrome

Pulmonary Manifestations of Primary Immunodeficiency Disorders in Children

Hemoptysis in a Patient with Elevated Immunoglobulin E

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