دورية أكاديمية

Chinese Erdheim-Chester disease: clinical-pathology-PET/CT updates.

التفاصيل البيبلوغرافية
العنوان: Chinese Erdheim-Chester disease: clinical-pathology-PET/CT updates.
المؤلفون: Ding H; Endocrinology and , the First Affiliated Hospital of Sun Yat-sen University , Guangzhou, 510080 , People's Republic of China., Li Y; Department of Pathology , the First Affiliated Hospital of Sun Yat-sen University , Guangzhou, 510080 , People's Republic of China., Ruan C; Department of Endocrinology and Metabolism , Longyan People Hospital , Longyan, 364000 , People's Republic of China ., Gao Y; Department of General Practice , Community Health Center of Qianjin Street, Tianhe District , Guangzhou, 510660 , People's Republic of China., Wang H; Hematology, and Nuclear Medicine , the First Affiliated Hospital of Sun Yat-sen University , Guangzhou, 510080 , People's Republic of China., Zhang X; Nuclear Medicine , the First Affiliated Hospital of Sun Yat-sen University , Guangzhou, 510080 , People's Republic of China., Liao Z; Endocrinology and , the First Affiliated Hospital of Sun Yat-sen University , Guangzhou, 510080 , People's Republic of China.
المصدر: Endocrinology, diabetes & metabolism case reports [Endocrinol Diabetes Metab Case Rep] 2015; Vol. 2015, pp. 150055. Date of Electronic Publication: 2015 Sep 03.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Bioscientifica Country of Publication: England NLM ID: 101618943 Publication Model: Print-Electronic Cited Medium: Print ISSN: 2052-0573 (Print) Linking ISSN: 20520573 NLM ISO Abbreviation: Endocrinol Diabetes Metab Case Rep Subsets: PubMed not MEDLINE
أسماء مطبوعة: Original Publication: Bristol : Bioscientifica, [2013]-
مستخلص: Unlabelled: Erdheim-Chester disease (ECD), one type of systemic non-Langerhans cell histiocytosis, has been rarely seen and is characterized by the accumulation of foamy CD68+CD1a- histiocytes. We reported a case of ECD and reviewed the clinical features of 13 cases of ECD reported so far in China. A 53-year-old male was diagnosed with central diabetes insipidus in March 2014, followed by fever, splenomegaly and anemia in July 2014. His initial pituitary magnetic resonance imaging (MRI) revealed the absence of high signal at T1-weighted image in posterior pituitary without any lesion. A further positron emission tomography/computer tomography (PET/CT) images showed elevated metabolic activity of (18)F-2-fluro-D-deoxy-glucose (FDG) and low (13)N-NH3 uptake in the posterior pituitary, and multi-organ involvement. Biopsy at right femur lesion revealed that granulomatous infiltration of foamy histiocytes and Touton giant cells surrounded by fibrosis tissues. Immunohistochemistry stain was positive for CD68, negative for CD207/Langerin and S-100. The diagnosis of ECD was confirmed and the treatment with pegylated interferon was effective. ECD was a possible immune-related disorder concluding from the IgG4 immunohistochemistry results. We summarized the pathological manifestations for ECD and its differential diagnosis from Langerhans cell histiocytosis (LCH) and Rosai-Dorfman disease (RDD). ECD should be considered by both pathologists and clinicians in the differential diagnosis when central diabetes insipidus is accompanied with multi-organ involvement, especially skeletal system involvement, or recurrent fever.
Learning Points: ECD should be considered when central diabetes insipidus is accompanied with multisystem involvement, especially symmetric/asymmetric bone lesions, or recurrent fever.PET/CT scanning was helpful for locating pituitary lesion, discovering multiple system involvement and indicating the biopsy sites.Conducting proper immunohistochemistry stains was important for diagnosing ECD. ECD might be correlated with immune disorder.
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تواريخ الأحداث: Date Created: 20151104 Date Completed: 20151103 Latest Revision: 20200930
رمز التحديث: 20221213
مُعرف محوري في PubMed: PMC4626652
DOI: 10.1530/EDM-15-0055
PMID: 26527559
قاعدة البيانات: MEDLINE
الوصف
تدمد:2052-0573
DOI:10.1530/EDM-15-0055