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Chester Erdheim disease

Mohammad Adawi, Bishara Bisharat, Abdalla Bowirrat
BACKGROUND: Erdheim-Chester disease (ECD) is an uncommon aggressive, multisystem form of non-Langerhans' cell histocytosis, which was firstly reported by Jakob Erdheim and William chester in 1930. The disease pathological features encompass an aberrant multiplication, overproduction and accumulation of white blood cells called histiocytes within multiple tissues and organs. Herein, we present a case of ECD owing to the rarity of this disease (roughly 550 cases have been described in the literature to date)...
October 2016: Medicine (Baltimore)
Atman A Dave, Susan E Gutschow, Christopher M Walker
Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytosis that may be clonal and inflammatory in origin. The hallmark of the disease is infiltration of various organ systems by CD68+/CD1a- histiocytes containing foamy lipid-laden inclusions. The manifestations and course of the disease are variable and depend on the organ systems that are affected. Patients may be asymptomatic or may develop life-threatening complications, including myocardial infarction. The most common clinical manifestation is lower extremity bone pain...
September 13, 2016: Curēus
Sang Gyu Park, Dong Hoon Baek, Kyu Min Lee, Seo Young Oh, Do Youn Park, Sangjeong Ahn, Nari Shin, Geun Am Song
No abstract text is available yet for this article.
October 13, 2016: Journal of Digestive Diseases
C M Rice, C A Hall, P McCoubrie, S A Renowden, N Cohen, N J Scolding
We report a case of Erdheim-Chester disease (ECD) with a 25-year history following initial presentation with diabetes insipidus and brainstem involvement. The exceptionally long history is particularly notable, given that ECD is a life-threatening disorder and there is a recognised association between central nervous system involvement and poor outcome. The case is a timely reminder of the presenting features of the condition, given the emergence of potential new treatment options.
October 4, 2016: BMJ Case Reports
Davide Gianfreda, Alessandro A Palumbo, Enrica Rossi, Lorenzo Buttarelli, Gaia Manari, Chiara Martini, Massimo De Filippo, Augusto Vaglio
No abstract text is available yet for this article.
September 23, 2016: Blood
Archana George Vallonthaiel, Asit Ranjan Mridha, Shivanand Gamanagatti, Manisha Jana, Mehar Chand Sharma, Shah Alam Khan, Sameer Bakhshi
Erdheim-Chester disease (ECD) is an uncommon, non-familial, non-Langerhans cell histiocytosis, which involves skeletal system and soft tissue usually in middle aged and elderly patients. The characteristic radiologic features include bilateral, symmetric cortical osteosclerosis of the diaphyseal and metaphyseal parts of the long bones, or bilateral symmetrically abnormal intense (99m)Technetium labelling of the metaphyseal-diaphyseal region of the long bones, and computed tomography scan findings of "coated aorta" or "hairy kidneys"...
August 28, 2016: World Journal of Radiology
Brandon T Larsen, Maxwell L Smith, Brett M Elicker, Jessica M Fernandez, Guillermo A Arbo-Oze de Morvil, Carlos A C Pereira, Kevin O Leslie
Context .- Idiopathic pulmonary fibrosis (IPF) is a distinctive clinicopathologic entity and the most common form of progressive diffuse lung scarring in older adults. Idiopathic pulmonary fibrosis manifests histopathologically as the usual interstitial pneumonia pattern. The usual interstitial pneumonia pattern is distinguished by geographically and temporally heterogeneous fibrosis that is peripherally accentuated, often with honeycombing and traction bronchiectasis. Idiopathic pulmonary fibrosis is not the only disease that leads to end-stage lung fibrosis, however, and several other entities may also cause advanced fibrosis...
September 15, 2016: Archives of Pathology & Laboratory Medicine
Elisabetta Ferrero, Angelo Corti, Julien Haroche, Daniela Belloni, Barbara Colombo, Alvise Berti, Giulio Cavalli, Corrado Campochiaro, Antonello Villa, Fleur Cohen-Aubart, Zahir Amoura, Claudio Doglioni, Lorenzo Dagna, Marina Ferrarini
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis (LCH) characterized by tissue infiltration with CD68(+) foamy histiocytes. TNF-related chronic inflammation and mutations in the MAP kinase signaling pathway in histiocytes are recognized as the two major pathogenic events. Among pleomorphic clinical manifestations, cardiovascular involvement is frequent and prognostically relevant. Evaluation of ECD clinical course and response to treatment is, however, still challenging. Taking advantage of the two largest cohorts of ECD patients worldwide, we investigated the relevance and the potential of circulating Chromogranin A (CgA), a pro-hormone involved in cardiovascular homeostasis and inflammation, as a biomarker of response to therapy in ECD...
July 2016: Oncoimmunology
Sarah S Pinney, Richard R Jahan-Tigh, Susan Chon
Non-Langerhans cell histiocytosis (NLCH) is a histiocyte disorder comprised of dermal dendritic histiocytes with a characteristic staining pattern. Erdheim-Chester disease (ECD) is a subset of NLCH in which patients experience bone pain with corresponding changes on imaging. In addition, these patients show other evidence of systemic involvement, which can also be identified with imaging. This disease can occasionally present with cutaneous findings. We present a case of generalized eruptive histiocytosis (GEH), misdiagnosed as ECD, found to have an NTRK1 gene rearrangement...
2016: Dermatology Online Journal
Na Niu, Xinxin Cao, Ruixue Cui
Cervical and lumbosacral nerve roots and plexus involvement in Erdheim-Chester disease (ECD) is rare. A 50-year-old man with progressive edema in bilateral lower extremities, numbness, weakness, and muscle atrophy of upper limb for 2 years had been misdiagnosed of neuritis or cervical spondylosis until he presented bone pain. In addition to bilateral symmetric involvement in long bones, bilateral hypermetabolism in cervical and lumbosacral nerve roots was found by FDG PET/CT scan. ECD was diagnosed by bone biopsy...
November 2016: Clinical Nuclear Medicine
Melissa Matzumura, Javier Arias-Stella, James E Novak
Erdheim-Chester disease (ECD) is a rare, xanthogranulomatous, non-Langerhans cell histiocytosis with frequent systemic involvement. Although the diagnosis is based on characteristic histological and radiological findings, its identification can be challenging because of its heterogeneous presentation. Osteosclerosis of long bones, often associated with bone pain, is the most common initial manifestation, followed by extraskeletal manifestations in approximately 50% of cases. There is no standard treatment for ECD, although recommendations have been made on the basis of small studies...
July 2016: Journal of Investigative Medicine High Impact Case Reports
Daniel Vargas, J Caleb Richards, Daniel Ocazionez, Arlene Sirajuddin, Lorna Browne, Carlos S Restrepo
OBJECTIVE: The objectives of this article are: (1) to review common and rare manifestations of systemic and pulmonary Langerhans cell histiocytosis, Rosai-Dorfman disease, Erdheim-Chester disease and juvenile xanthogranuloma; (2) to provide the reader with important pathologic, epidemiologic and clinical features of these diseases. CONCLUSION: The histiocytoses are a diverse group of diseases which typically manifest with multiorgan involvement. Understanding the pathologic, epidemiologic, and clinical features of these entities can help the radiologist suggest an accurate diagnosis of histiocytosis when typical imaging features are encountered...
September 7, 2016: British Journal of Radiology
Stéphane Mathis, Gaëlle Godenèche, Julien Haroche, Serge Milin, Adrien Julian, Aline Berthomet, Clément Baron, Paola Palazzo, Jean-Philippe Neau
BACKGROUND: Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis. This inflammatory myeloid neoplasm is frequently complicated by neurological symptoms, but stroke is an exceptional manifestation of this disease. METHODS: We report the case of a 59-year-old woman who presented a vertebrobasilar stroke secondary to infiltration and severe stenosis of the basilar artery, improved after interferon-alpha therapy. We performed a review of the relevant literature and reported the few other cases described...
September 2016: Medicine (Baltimore)
S Wang, C P Ren, Y Li, B B Li, C P Xiao, F Li, J L Cheng
OBJECTIVE: To investigate the imaging features of Erdheim-Chester. METHODS: A total of 7 patients with ECD confirmed by pathology were analyzed retrospectively.And the DR(n=5), CT(n=6, 3 patients underwent enhanced scan), MR(n=4, 2 patients underwent enhanced scan) and SPECT (n=3) features were observed. RESULTS: Among 7 cases of ECD, four cases of DR images showed luffa cystic changes.Five cases of CT images showed bone dissolved, four showed peripheral localized sclerosis, one showed symmetric sclerosis, No enhancement was found on CT scan...
August 16, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Eli L Diamond, Omar Abdel-Wahab, Benjamin H Durham, Ahmet Dogan, Neval Ozkaya, Lynn Brody, Maria Arcila, Christian Bowers, Mark Fluchel
No abstract text is available yet for this article.
August 17, 2016: Blood
Jing-Shi Wang, Zhao Wang
Erdheim-Chester Disease (ECD) is a rare form of non Langerhans' cell histiocytosis. The etiology of ECD is unknown yet. The disease is characterized by the infiltration of lipid-laden histiocytes with foamy to bones and various organs. The heterogeneous manifestations of ECD vary among different individuals. This results in a presentation that may vary from an indolent focal disease to a life threatening organ failure. This review focuses on the etiology, clinical presentation, diagnostic criteria and treatment of Erdheim-Chester disease (ECD)...
August 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
Y P Chen, X N Jiang, J P Lu, H Zhang, X Q Li, G Chen
OBJECTIVE: To investigate the clinicopathologic features, diagnosis and differential diagnosis of extranodal Rosai-Dorfman disease(RDD)of the breast. METHODS: Twelve cases of extranodal RDD of the breast were analyzed using hematoxylin-eosin stain and immunohistochemical staining.The morphological features and immunophenotype were observed by light microscopy, accompanied with a review of the literature. RESULTS: Twelve cases of extranodal RDD of the breast are presented...
August 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Jonathan Nadjiri, Klaus Woertler, Katja Specht, Norbert Harrasser, Andreas Toepfer
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with a broad spectrum of organ manifestations, presenting with characteristic radiological and histological findings. Soft tissue manifestations (xanthogranulomas) have been reported to be most commonly found in the region of the orbits. We report bilateral Achilles tendon xanthogramlomas in a 36-year-old male with biopsy-proven and B-RAF V600E-positive ECD. Although rare, ECD should be considered in the differential diagnosis of intratendinous masses...
October 2016: Skeletal Radiology
Pengcheng Zhu, Naping Li, Lu Yu, Mariajose Navia Miranda, Guoping Wang, Yaqi Duan
Histiocytosis is an uncommon disease characterized by excessive accumulation of histiocytes. Here, we report a rare case of non-Langerhans-cell histiocytosis in a 51-year-old woman who presented with severe symptoms of pericardial effusion. Radiologic investigation also detected multiple bone (lower limbs, vertebrae, ribs, and ilium) lesions. Resected pericardium showed abundant mono- or multi-nucleated non-foamy histiocytes (CD68+/CD163+/S-100+/CD1α-/Langerin-) in a fibroinflammatory background. The histiocytes demonstrated emperipolesis of lymphocytes, a hallmark feature of Rosai-Dorfman Disease (RDD)...
July 28, 2016: Cancer Research and Treatment: Official Journal of Korean Cancer Association
Xiaoyan Liao, John A Thorson, Tudor Hughes, John C Nguyen, Huan-You Wang, Grace Y Lin
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by xanthogranulomatous infiltration of foamy histiocytes frequently involving bone and other organ systems. We herein report a unique case of ECD discovered incidentally in an explanted liver in a 65-year-old male with end-stage liver disease secondary to hepatitis C cirrhosis. Histological examination and immunohistochemical studies in the explanted liver revealed prominent foamy histiocytes that were CD68 positive, but CD1a and S100 negative...
September 2016: Pathology, Research and Practice
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