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Langerhans histiocytosis

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https://www.readbyqxmd.com/read/28421232/clinical-spectrum-quality-of-life-braf-mutation-status-and-treatment-of-skin-involvement-in-adult-langerhans-cell-histiocytosis
#1
Etienne Crickx, Jean-David Bouaziz, Gwenaël Lorillon, Mathilde de Menthon, Florence Cordoliani, Emmanuell Bugnet, Martine Bagot, Michel Rybojad, Samia Mourah, Abdellatif Tazi
Langerhans cell histiocytosis is a rare histiocytic disorder for which skin involvement and management are poorly described in adults. The aim of this retrospective monocentric study in a national reference centre is to describe the clinical characteristics, quality of life, BRAF mutation status and outcomes of skin involvement in adult patients with Langerhans cell histiocytosis. Twenty-five patients (14 females, mean age 47 years) were included, with a median follow-up of 33 months (range 4-420 months). Patients experienced poor dermatological quality of life despite low body surface involvement...
April 19, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28417851/nuclear-medicine-techniques-in-pediatric-bone-imaging
#2
REVIEW
Laura A Drubach
An important mandate when imaging pediatric patients is the reduction of radiation exposure to the lowest possible level consistent with good quality diagnostic imaging, so individual selection of radiopharmaceutical dosage and imaging technique is essential. Although the logistics of image acquisition and the approach to interpretation mirror the more common use in adults, the challenges of imaging young children typically require greater attention to patient preparation, positioning, and supervision during imaging, with the use of parental or family engagement in the procedure, gentle restraint, and occasionally the need for sedation...
May 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28413819/multisystemic-langerhans-cell-histiocytosis-in-an-adult
#3
Marie-Valerie Hegemann, Stephan Schreml
No abstract text is available yet for this article.
March 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28407623/fine-needle-aspiration-of-langerhans-cell-histiocytosis-a-cytopathologic-study-of-37-cases
#4
Jen-Fan Hang, Momin T Siddiqui, Syed Z Ali
OBJECTIVE: Langerhans cell histiocytosis (LCH) is an uncommon neoplasm of dendritic cells and predominantly occurs in children and young adults. The study aims to evaluate cytopathologic features and current diagnostic concepts in a large series of LCH on fine needle aspiration (FNA). STUDY DESIGN: We retrospectively searched the pathology archives of The Johns Hopkins Hospital (JHH) and Emory University Hospital (EUH) to identify all FNA cases diagnosed as LCH in a period of 21 years...
April 14, 2017: Acta Cytologica
https://www.readbyqxmd.com/read/28406136/pulmonary-langerhans-cell-histiocytosis-case-with-diabetes-insipidus-and-tuberculosis
#5
E Ugurlu, G Altinisik, U Aydogmus, F Bir
A 19-year-old male patient was observed due to having central diabetes insipidus (DI) for five years. He had a history of smoking 5-10 cigarettes a day for two years, but stopped smoking from the last month. The computerized tomography revealed thin-walled cystic lesions in different sizes more dominantly in the upper lobes and consolidated areas in the left upper and lower lobes. The wedge resection from the right lower lobe revealed pulmonary langerhans cell histiocytosis. Follow-up acid-fast bacteria (AFB) examinations revealed (+++) and antituberculous treatment was started...
April 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28403515/an-aggressive-multifocal-primary-cns-histiocytosis-with-ptpn11-shp2-mutation
#6
Qi Zhang, Areej Shibani, Bekim Sadikovic, Christopher J Howlett, Lee-Cyn Ang
Primary histiocytic tumors of the CNS are rare. The current WHO classification (2016) included 5 entities: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and histiocytic sarcoma (HS) (1). The diagnosis usually is made based on the tumor differentiation as to the counterpart in normal histiocyte development. This article is protected by copyright. All rights reserved.
April 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28398921/hemosiderotic-juvenile-xanthogranuloma
#7
Angel Fernandez-Flores, Ina Nicklaus, Fiona Browne, Isabel Colmenero
Juvenile xanthogranuloma is a non-Langerhans cell lesion mostly limited to the skin but occasionally presenting in extracutaneous locations or associated with systemic conditions. Lesions need to be distinguished mainly from dermatofibroma, xanthoma, Langerhans cell histiocytosis, or reticulohistiocytoma. Herein, we present a hemosiderotic variant of juvenile xanthogranuloma in a 12-year-old girl, which we have not found described in literature. The lesion presented at the back of the scalp as a slowly growing yellowish polypoid lesion showing occasional bleeding...
March 16, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28396615/bone-pathologic-fracture-revealing-an-unusual-association-coexistence-of-langerhans-cell-histiocytosis-with-rosai-dorfman-disease
#8
Boubacar Efared, Asmae Mazti, Badarou Chaibou, Gabrielle Atsame-Ebang, Ibrahim Sory Sidibé, Layla Tahiri, Fatimazahra Erregad, Nawal Hammas, Abdelmajid El Mrini, Hinde El Fatemi, Laila Chbani
BACKGROUND: The coexistence of Rosai-Dorfman disease (RDD) with Langerhans cell histiocytosis (LCH) is very rare, as to date only 17 cases have been reported in the english literature. The pathophysiology of this uncommon co-occurrence still remains enigmatic and a subject of various speculations. CASE PRESENTATION: We report a case of a 30-year-old female patient who presented with a pathologic fracture of the left proximal femur. Her medical history was unremarkable, there were no fever, skin lesions, lymphadenopathy or other organomegaly at physical examination...
2017: BMC Clinical Pathology
https://www.readbyqxmd.com/read/28377643/langerhans-cell-histiocytosis-of-the-cervical-spine-in-an-adult-patient-case-report-and-review-of-the-literature
#9
Ines Vielgut, Bernadette Liegl-Atzwanger, Gerhard Bratschitsch, Andreas Leithner, Roman Radl
Langerhans-cell histiocytosis (LCH) is a rare, benign bone tumor, usually occurring in children and younger adults under 20 years old. Only a few cases of solitary bone lesions of the adult spine are reported in literature, therapeutic guidelines or treatment regimens for lesions of the adult spine are not established yet to our knowledge.
June 2017: Journal of Orthopaedics
https://www.readbyqxmd.com/read/28365280/-self-healing-lesions-in-langerhans-cell-histiocytosis
#10
Fátima Tous-Romero, Raquel Aragón-Miguel, Carlos Zarco-Olivo, Sara Palencia-Pérez
No abstract text is available yet for this article.
March 29, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28361364/spontaneous-and-complete-regeneration-of-a-vertebra-plana-after-surgical-curettage-of-an-eosinophilic-granuloma
#11
Francesca Di Felice, Fabio Zaina, Sabrina Donzelli, Stefano Negrini
PURPOSE: The eosinophilic granuloma is a unifocal or multifocal Langerhans cell histiocytosis characterized by an expanding proliferation of Langerhans cells in bones. Skeletal LCH is a rare condition, and vertebral regeneration in cases of vertebral body collapse is even rarer. We report the case of a girl with spontaneous complete healing. METHODS AD RESULTS: A 3-year-old girl was referred for nighttime back pain, with no fever and no neurologic signs. Within a few days, she developed sudden painful restriction of all spine movements...
March 30, 2017: European Spine Journal
https://www.readbyqxmd.com/read/28360976/langerhans-cell-histiocytosis-masquerading-as-acute-appendicitis-case-report-and-review
#12
Mohammad M Karimzada, Michele N Matthews, Samuel W French, Daniel DeUgarte, Dennis Y Kim
Langerhans cell histiocytosis (LCH) is a rare syndrome characterized by unifocal, multifocal unisystem, or disseminated/multi-system disease that commonly involves the bone, skin, lymph nodes, pituitary, or sometimes lung (almost exclusively in smokers) causing a variety of symptoms from rashes and bone lesions to diabetes insipidus or pulmonary infiltrates. We present a previously unreported case of gastrointestinal LCH as well as a novel characteristic lesion affecting the colon of a young woman who presented with signs and symptoms mimicking acute on chronic appendicitis...
March 16, 2017: World Journal of Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28353530/cranium-penetrating-mass-detected-by-ultrasound-expedited-management-of-langerhans-cell-histiocytosis
#13
Brian Morgan, Hsien Lee Lau, Sharon Yellin
A 6-year-old boy presented to the pediatric emergency department with a unilateral 5 × 3-cm superficial mass on the postauricular region growing for 1 month. Point-of-care ultrasound was used to evaluate the mass, which revealed a complex cystic mass penetrating the temporal bone. After confirmatory magnetic resonance imaging, the patient was transferred for neurosurgical evaluation, and the tumor was excised. Pathology revealed Langerhans cell histiocytosis.
April 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28329596/coalescing-nodules-on-the-trunk-of-an-infant-juvenile-xanthogranuloma-en-plaque
#14
Jennifer T Hau, Kathy Langevin
Juvenile xanthogranuloma (JXG) is a benign, non-Langerhans cell histiocytosis, which commonly manifests as a solitary papule or nodule in infants and young children. However, other less common clinical presentations have been reported including clustered, agminated or plaque-like. It is important to recognize these variants, as diagnosis is often clinical. We highlight a case of juvenile xanthogranuloma presenting as nodules on the trunk of an infant that gradually coalesced to form a larger plaque.
October 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28326824/osteopontin-its-potential-role-in-cancer-of-children-and-young-adults
#15
Gabrielle Karpinsky, Aleksandra Fatyga, Malgorzata Anna Krawczyk, Madeleine Chamera, Natalia Sande, Dagmara Szmyd, Ewa Izycka-Swieszewska, Ewa Bien
OBJECTIVE: Osteopontin (OPN) is aglyco-phosphoprotein, involved in tissue remodeling, inflammation and boneresorption. In various adult neoplasms OPN was shown to correlate with cancer progression, invasiveness and metastasis. AIM: to define the role of OPN in malignancies of children and young adults. MATERIAL AND METHODS: a structured PubMed and Google Scholar literature analysis based on reports published in English between I'1995 and XII'2015...
March 14, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/28318197/-generalized-eruptive-histiocytosis-juvenile-xanthogranuloma-clinical-spectrum-in-a-pediatric-patient
#16
Ruth Alperovich, Pedro T Grassino, Raúl Asial, Luis Pasteris, María Del Carmen Boente
Both, generalized eruptive histiocytosis and juvenile xanthogranuloma are dendritic histiocytic disorders (also known as non-Langerhans cells histiocytosis) that share clinicopathological and immunohistiochemical characteristics. We present a 3-year-old female patient with skin lesions that were clinically compatible with generalized eruptive histiocytosis, confirmed by histopathological and immunohistochemical studies. During her development the disorder compromised the central nervous system, and surgical intervention of one symptomatic lesion was needed...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28315918/fluorrine-18-fluorodeoxyglucose-positron-emission-tomography-computed-tomography-of-adult-liver-langerhans-cell-histiocytosis
#17
Hengshan Ji, Jiang Wu, Guifen Yang, Chuanjin Sun, Hong Zhu, Longjiang Zhang
OBJECTIVE: Adult liver Langerhans cell histiocytosis (LCH) is an extremely rare desease. This paper reports a 40 years old male patient who was diagnosed as liver LCH though ultrasound-guided liver biopsy. The initial Fluorrine-18- fluorodeoxyglucose positron emission tomography/ computed tomography ((18)F-FDG PET/CT) showed multiple nodular low-density lesions in liver without obvious elevated (18)F-FDG uptake. Four years later, the follow-up (18)F-FDG PET/CT showed the liver multiple lesions with slightly elevated (18)F-FDG uptake...
January 2017: Hellenic Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/28302693/langerhans-cell-histiocytosis-in-a-patient-with-hairy-cell-leukemia-a-tale-of-divergence
#18
Sanam Loghavi, Joseph D Khoury
No abstract text is available yet for this article.
March 16, 2017: Blood
https://www.readbyqxmd.com/read/28301225/optimal-diagnostic-yield-achieved-with-on-site-pathology-evaluation-of-fine-needle-aspiration-assisted-core-biopsies-for-pediatric-osseous-lesions-a-single-center-experience
#19
Kalyani Patel, Darryl Kinnear, Norma M Quintanilla, John Hicks, Eumenia Castro, Choladda Curry, John Dormans, Daniel J Ashton, J Alberto Hernandez, Hao Wu
CONTEXT: - Image-guided, fine-needle aspiration-assisted core needle biopsy with an on-site evaluation by a pathologist (FNACBP) of osseous lesions is not a common practice in pediatric institutions. OBJECTIVES: - To evaluate the diagnostic adequacy and accuracy of FNACBP for pediatric osseous lesions and to compare the adequacy with procedures that do not use fine-needle aspiration. DESIGN: - Six-year, retrospective review of 144 consecutive children biopsied for osseous lesions with and without fine-needle aspiration assistance...
March 16, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28300446/a-rare-case-of-solitary-giant-congenital-juvenile-xanthogranuloma
#20
Catherine Vignault, Émilie Bourgeault, Éric Gagné, Janie Bujold
Juvenile xanthogranuloma (JXG) is the most frequent form of non-Langerhans cell histiocytosis. We present a case of giant congenital JXG in a 7-week-old boy, who had a firm and incompressible lesion, measuring 3 × 4 cm in diameter, on his right flank. The clinical appearance of the lesion and the ultrasound results suggested a vascular tumor, such as a hemangioma. Histology confirmed a JXG, although there was an absence of Touton cells, which are usually pathognomonic of JXG. In light of these findings, it would be important to include JXG in the differential diagnosis of congenital tumours, particularly vascular lesions...
February 1, 2017: Journal of Cutaneous Medicine and Surgery
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