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https://www.readbyqxmd.com/read/27908938/a-rare-case-of-extensive-cranial-langerhans-cell-histiocytosis-synchronously-presenting-as-otitis-externa-and-giant-cell-arteritis
#1
Adnan Darr, Zahir Mughal, Thomas Martin
Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unpredictable clinical course and varied modes of presentation. The spectrum of presentation is wide, ranging from isolated eosinophilic granulomas to multiple lesions and diffuse systemic involvement. We present the case of a 52-year-old man, who presented with an 8-week history of worsening otalgia and superficial temporal tenderness attributed to otitis externa within the community and subsequently giant cell arteritis. Computed tomography and magnetic resonance imaging were undertaken due to atypical features, which demonstrated bony destruction within the right greater wing of the sphenoid, squamous part of temporal and mastoid bone, with middle cranial fossa communication...
December 1, 2016: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/27900869/-evaluation-of-five-years-of-treatment-of-erdheim-chester-disease-with-anakinra-case-report-and-overview-of-literature
#2
Zdeněk Adam, Hana Petrášová, Zdeněk Řehák, Renata Koukalová, Marta Krejčí, Luděk Pour, Eva Vetešníková, Aleš Čermák, Sabina Ševčíková, Petr Szturz, Zdeněk Král, Jiří Mayer
: Erdheim-Chester disease is a histiocytic neoplasm of diseases from the group of non-Langerhans-cell histiocytoses, formed by infiltrates of foamy histiocytes. These pathological histiocytes produce pro-inflammatory cytokines. Therefore Erdheim-Chester disease is called inflammatory histiocytary neoplasm. The disease is accompanied by clinical symptoms of systemic inflammatory response, i.e. B symptoms. Imaging examinations detect typical osteosclerotic changes affecting diaphyses and metaphyses of the lower long bones and fibrotic changes which affect the aorta wall and the vessels leading from it...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27900055/langerhans-cell-sarcoma-originating-from-left-knee-subcutaneous-tissue-a-case-report-and-literature-review
#3
Yaonan Zhang, Zongyang Qu, Fang Fang
Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with notably malignant cytological features. Reports of LCS are sparsely available in English literature; to the best of our knowledge, only 55 cases have been reported. The present study reports a case of LCS originating from subcutaneous tissue of the left knee in a 75-year-old man. The diagnosis of LCS was supported by the results of magnetic resonance imaging, histological and immunohistochemical studies. The tumor began to metastasize to inguinal lymph nodes and eventually involved multiple organs...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27898473/histopathological-and-clinical-findings-in-cutaneous-manifestation-of-erdheim-chester-disease-and-langerhans-cell-histiocytosis-overlap-syndrome-associated-with-the-brafv600e-mutation
#4
Julia Liersch, J Andrew Carlson, Jörg Schaller
The overlap of Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) is more common than it was generally accepted. Both diseases seem to be linked by a mutation in oncogenic BRAFV600E, probably an early event which occurs in bone marrow progenitor cells. In this article are described the clinical and histological findings in 2 cases of ECD-LCH overlap syndrome bearing the BRAFV600E mutation in both ECD and LCH lesions in bone and skin. In one case, lesions of ECD and LCH were situated directly site-to-site in the same bone section leading to the assumption of a common myeloid precursor cell for these diseases...
November 23, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27895996/label-free-fast-3d-coherent-imaging-reveals-pancreatic-islet-micro-vascularization-and-dynamic-blood-flow
#5
Corinne Berclaz, Daniel Szlag, David Nguyen, Jérôme Extermann, Arno Bouwens, Paul J Marchand, Julia Nilsson, Anja Schmidt-Christensen, Dan Holmberg, Anne Grapin-Botton, Theo Lasser
In diabetes, pancreatic β-cells play a key role. These cells are clustered within structures called islets of Langerhans inside the pancreas and produce insulin, which is directly secreted into the blood stream. The dense vascularization of islets of Langerhans is critical for maintaining a proper regulation of blood glucose homeostasis and is known to be affected from the early stage of diabetes. The deep localization of these islets inside the pancreas in the abdominal cavity renders their in vivo visualization a challenging task...
November 1, 2016: Biomedical Optics Express
https://www.readbyqxmd.com/read/27894453/usual-and-unusual-manifestations-of-familial-hemophagocytic-lymphohistiocytosis-and-langerhans-cell-histiocytosis
#6
REVIEW
Craig Erker, Paul Harker-Murray, Julie-An Talano
Familial hemophagocytic lymphohistiocytosis (FHL) and Langerhans cell histiocytosis (LCH) are histiocytic diseases that occur most commonly in young children. Improvements in recognition and treatment have been substantial for both diseases in the past decade, although early and late morbidity continue to be major concerns. These two diagnoses behave differently, although the clinical spectra for both diseases are diverse and can lead to confusion and delays in diagnosis and treatment. This article focuses on the clinical and genetic spectrum of FHL as well as the clinical and treatment variations of LCH...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27892930/an-optimized-protocol-for-purification-of-functional-islets-of-langerhans
#7
Youakim Saliba, Jules-Joel Bakhos, Tarek Itani, Nassim Farès
Islets of Langerhans and β-cell isolation constitute routinely used cell models for diabetic research, and refining islet isolation protocols and cell quality assessment is a high priority. Numerous protocols have been published describing isolate of islets, but often rigorous and systematic assessment of their integrity is lacking. Herein, we propose a new protocol for optimal generation of islets. Pancreases from mice and rats were excised and digested using a low-activity collagenase solution and islets were then purified by a series of sedimentations and a Percoll gradient...
November 28, 2016: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/27890297/digesting-the-role-of-bone-marrow-macrophages-on-hematopoiesis
#8
REVIEW
Esther Heideveld, Emile van den Akker
Tissue resident macrophages are found in various tissues like Langerhans cells in the skin or alveolar macrophages in the lung, and their main function is to regulate organ homeostasis. They have also been observed in the bone marrow and these cells in particular have been gaining importance in recent years as they are key players in hematopoiesis. However, as the characterization and classification of these putatively different bone marrow resident macrophages is far from established there is a need to generate an overview of tissue resident macrophages of the bone marrow...
November 17, 2016: Immunobiology
https://www.readbyqxmd.com/read/27889325/-langerhans-cell-histiocytosis-and-erdheim-chester-disease-a-continuity
#9
S Parreau, J Haroche, I Pommepuy, J F Emile, J C Bourras, F Archambeaud
INTRODUCTION: Erdheim-Chester disease and langerhans cell histiocytosis are two rare diseases separate on clinical, radiological and histological aspects. However, several cases involving both entities have been described. OBSERVATION: A 70-year-old man had a central diabetes insipidus, xanthelasmas, retroperitoneal fibrosis and osteosclerosis of the legs suggestive of Erdheim-Chester disease. Bone biopsy showed langerhans cell histiocytosis CD1a positive with the presence of BRAF V600E mutation...
November 23, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27886798/multicentric-reticulohistiocytosis-rheumatology-perspective
#10
REVIEW
Bahtiyar Toz, Nesimi Büyükbabani, Murat İnanç
Multicentric reticulohistiocytosis (MRH) is a rare, multisystemic non-Langerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. There are no specific laboratory findings for MRH. Diagnosis is based on clinical findings and skin or synovial biopsy results. There is currently no consensus for the treatment of MRH. Here, we review the differential diagnosis and treatment options of MRH from the rheumatologist's perspective. We also report an index case of MRH associated with Sjögren's syndrome and pulmonary embolism...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27878497/role-of-tissue-specific-stem-and-progenitor-cells-in-the-regeneration-of-the-pancreas-and-testicular-tissue-in-diabetic-disorders
#11
E G Skurikhin, A V Pakhomova, N N Ermakova, O V Pershina, V A Krupin, E S Pan, A I Kudryashova, L A Ermolaeva, A M Dygai
Using the model of hypogonadism in C57Bl/6 male mice, we showed that injection of streptozotocin to newborn animals and high-fat diet induced serum IFN-γ and IL-17 elevation, glucose metabolism disturbances, insulin resistance, destructive changes of the Langerhans islets (deficit of PDX1(+)β cells), while the number of oligopotent β cell precursors (CD45(-)TER119(-)CD133(+)CD49f(low)) increased. Diabetes played the role of an inducer of testicular tissue inflammation (pan-hemopoietic cell infiltration, increase of IL-2, IL-17, and IL-23 content) and reproductive system disturbances in mice (decrease in free testosterone concentration, suppression of spermatogenesis, and infertility)...
November 23, 2016: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/27867869/thyroid-langerhans-cell-histiocytosis-and-papillary-thyroid-carcinoma
#12
Rajab AlZahrani, Mohammed Algarni, Hadi Alhakami, Haia AlSubayea, Naif Alfattani, Mohammet Guler, Mohamed Satti
A 27-year-old female, married with two children, presented to our clinic with a 1-year history of thyroid swelling and pressure symptoms on lying backward and bilateral cervical lymphadenopathy. The patient was a known case of panhypopituitarism for 5 years. Comprehensive patient evaluation including FNAC with papillary thyroid cancer result then she underwent total thyroidectomy and bilateral neck dissection and final histologic examination confirmed papillary thyroid carcinoma in the background of lymphocytic thyroiditis, associated with Langerhans cell histiocytosis (LCH)...
October 2016: Gland Surgery
https://www.readbyqxmd.com/read/27865989/drug-delivery-across-intact-and-disrupted-skin-barrier-identification-of-cell-populations-interacting-with-penetrated-thermoresponsive-nanogels
#13
F Rancan, M Giulbudagian, J Jurisch, U Blume-Peytavi, M Calderón, A Vogt
Nanoscaled soft particles, such as nanogels, can be designed to incorporate different types of compounds and release them in a controlled and triggered manner. Thermoresponsive nanogels (tNG), releasing their cargo above a defined temperature, are promising carrier systems for inflammatory skin diseases, where the temperature of diseased skin differs from that of healthy skin areas. In this study a polyglycerol-based tNG with diameter of 156 nm was investigated for penetration and release properties upon topical application on ex vivo human skin with intact or disrupted barrier...
November 16, 2016: European Journal of Pharmaceutics and Biopharmaceutics
https://www.readbyqxmd.com/read/27865360/langerhans-cell-number-is-a-strong-and-independent-prognostic-factor-for-head-and-neck-squamous-cell-carcinomas
#14
Nadège Kindt, Géraldine Descamps, Imelda Seminerio, Justine Bellier, Jérôme R Lechien, Charles Pottier, Denis Larsimont, Fabrice Journé, Philippe Delvenne, Sven Saussez
OBJECTIVES: Head and neck squamous cell carcinomas (HNSCCs) exhibit great biological heterogeneity and relatively poor prognosis. Tobacco and alcohol consumption is involved in the cause of the majority of these cancers, but over the last several years, Human Papilloma Virus (HPV) infection has increased specifically in oropharyngeal cancers and become an additional risk factor. Here, we evaluated the number of Langerhans cells (LCs) in HNSCC and reporting its prognostic power in comparison to other risk factors...
November 2016: Oral Oncology
https://www.readbyqxmd.com/read/27853924/retrospective-analysis-of-nonendodontic-periapical-lesions-misdiagnosed-as-endodontic-apical-periodontitis-lesions-in-a-population-of-taiwanese-patients
#15
Hsun-Yu Huang, Yuk-Kwan Chen, Edward Cheng-Chuan Ko, Fu-Hsiung Chuang, Ping-Ho Chen, Ching-Yi Chen, Wen-Chen Wang
OBJECTIVE: We aimed to evaluate nonendodontic periapical lesions clinically misdiagnosed as endodontic periapical pathoses in a population of Taiwanese patients. MATERIALS AND METHODS: Cases (2000-2014) of histopathological diagnoses of nonendodontic periapical lesions were retrieved from all cases with a clinical diagnosis of radicular cyst, apical granuloma, or apical periodontitis in the institution. These cases were regarded as misdiagnosed nonendodontic periapical lesions, of which the types and frequencies, in addition to the demographic data, were determined...
November 16, 2016: Clinical Oral Investigations
https://www.readbyqxmd.com/read/27846659/the-non-langerhans-cell-histiocytoses-rare-histiocytoses-clinical-aspects-and-therapeutic-approaches
#16
C F Classen, M Minkov, T Lehrnbecher
Rare histiocytoses, also called non-Langerhans cell histiocytoses, include all proliferative disorders of histiocytes, macrophages and dendritic cells that are not classified as Langerhans cell histiocytosis (LCH) and do not belong to the hemophagocytic lymphohistiocytosis (HLH) group of diseases. Thus, the term includes numerous benign or malignant, localized or systemic, adult or pediatric diseases. The classification of the histiocytic disorders has been revised several times. Here, we follow the classification recently published by Jean Francois Emile and an international expert panel, defining subgroups of histiocytoses described as L-Group, C-Group, M-Group, R-Group, and H-Group, which stands for LCH-like, cutaneous or mucocutaneous, malignant, Rosai-Dorfman-Disease like and HLH like...
November 2016: Klinische Pädiatrie
https://www.readbyqxmd.com/read/27845884/central-diabetes-insipidus-clinical-profile-that-suggests-organicity-in-peruvian-children-lima-peru-2001-2013
#17
Miguel Angel De Los Santos, Carlos Manuel Del Águila, Maria Isabel Rojas, Juan Manuel Falen, Oswaldo Nuñez, Eliana Manuela Chávez, Oscar Antonio Espinoza, Paola Marianella Pinto, Martha Rosario Calagua
BACKGROUND: Central diabetes insipidus (CDI) is a heterogeneous disease caused by arginine vasopressin deficiency; its management implies a profound understanding of the pathophysiology and the clinical spectrum. The aim of the study was to describe the clinical characteristics that indicate organicity in children and adolescents with central diabetes insipidus treated at the Department of Endocrinology from The Child Health's Institute during 2001 to 2013. METHODS: Cross-sectional, retrospective study...
December 1, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27833773/a-fatal-case-of-congenital-langerhans-cell-histiocytosis-with-disseminated-cutaneous-lesions-in-a-premature-neonate
#18
Michio Inoue, Yoko Tomita, Tsuyoshi Egawa, Tomoaki Ioroi, Masaaki Kugo, Shinsaku Imashuku
Background. The outcome of neonates with congenital cutaneous Langerhans cell histiocytosis (LCH) is variable. Observations. We report a case of LCH in a female premature neonate born at 33-week gestation. She had disseminated cutaneous lesions, which consisted of hemorrhagic papules and vesicles, with sparse healthy skin areas, and the hands and feet were contracted with scarring and blackened. She was in respiratory failure although no apparent pulmonary or bone lesions on X-rays were noted. Skin biopsy confirmed a diagnosis of LCH due to observation of CD1a(+) Langerhans cells, which lacked expression of E-cadherin and CD56...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27823979/high-frequency-of-clonal-ig-and-t-cell-receptor-gene-rearrangements-in-histiocytic-and-dendritic-cell-neoplasms
#19
Wenting Huang, Tian Qiu, Linshu Zeng, Bo Zheng, Jianming Ying, Xiaoli Feng
The 2008 World Health Organization (WHO) diagnostic criteria of histiocytic and dendritic cell neoplasms from hematopoietic and lymphoid tissues no longer required the absence of clonal B-cell/T-cell receptor gene rearrangements. It is true that the clonal B-cell/T-cell receptor gene rearrangements have been identified in rare cases of histiocytic and dendritic cell neoplasms, such as those with or following lymphoma/leukemia or in some sporadic histiocytic/dendritic cell sarcomas, but the clonal features of such group of tumor are still not clear...
November 3, 2016: Oncotarget
https://www.readbyqxmd.com/read/27816346/-neuro-langerhans-cell-histiocytosis
#20
Loïc Le Guennec, Nadine Martin-Duverneuil, Karima Mokhtari, Maria Santiago-Ribeiro, Eléonore Bayen, Antoine Del Cul, Daniel Delgadillo, Aurélie Kas, Carine Courtillot, Julien Haroche, Fleur Cohen, Jean Donadieu, Khê Hoang-Xuan, Ahmed Idbaih
Langerhans cell histiocytosis (LCH) is a rare multisystemic disease. LCH is characterized by proliferation of myeloid progenitors with altered differentiation program and similar phenotypic features to epidermal dendritic cells termed Langerhans cell. LCH cells express CD1a+ and langerin and exhibit BRAF V600E mutation in ∼50% of cases. Neurological involvement or neuro-LCH is observed in 5 to 10% of cases. Three subtypes of neuro-LCH are individualized. The tumor type, accounting for 45% of neuro-LCH, affect mainly young adults...
November 2, 2016: La Presse Médicale
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