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Langerhans histiocytosis

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https://www.readbyqxmd.com/read/28329596/coalescing-nodules-on-the-trunk-of-an-infant-juvenile-xanthogranuloma-en-plaque
#1
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
#2
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
#3
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
#4
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...
March 20, 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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28295484/who-2016-classification-changes-and-advancements-in-the-diagnosis-of-miscellaneous-primary-cns-tumours
#8
Felix Sahm, David E Reuss, Caterina Giannini
This short review highlights significant changes and recent findings incorporated to varying extent in the WHO 2016 definition of a variety of tumours, including peripheral nerve sheath tumours, meningiomas, mesenchymal non-meningothelial tumours, melanocytic tumours, lymphomas and histiocytic tumours, germ cell tumours and non-neuroendocrine pituitary tumours. Most notable classification changes include: adding "hybrid nerve sheath tumours" to the spectrum of benign nerve sheath tumours; an updated definition of atypical meningioma (WHO grade II), including cases with brain invasion; recognizing dural solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) as a single tumour entity characterized by NAB2 and STAT6 gene fusions for which the term solitary SFT/HPC was chosen; recognizing that pituitary granular cell tumour, spindle cell oncocytoma, and pituicytoma all share nuclear expression of TTF-1, possibly representing a spectrum of a single nosological entity derived from posterior pituitary glial cells...
March 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28289985/perianal-lesions-in-children-an-updated-review
#9
Michelle Xu, Hannah Liu, Sharon Glick, Amor Khachemoune
Perianal lesions in children are common reasons for dermatology clinic visits and a well-defined approach to diagnosis and management is helpful to the practicing clinician. In this article, we review and update various etiologies of perianal lesions in the pediatric population, including infectious, papulosquamous, vascular, and neoplastic. We provide a standard initial approach to diagnosis and updates on current management. Infectious etiologies of perianal lesions discussed in this article include fungal, bacterial, parasitic, and viral...
March 13, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28289810/non-odontogenic-tumors-of-the-facial-bones-in-children-and-adolescents-role-of-multiparametric-imaging
#10
REVIEW
Minerva Becker, Salvatore Stefanelli, Anne-Laure Rougemont, Pierre Alexandre Poletti, Laura Merlini
Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin...
March 13, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28285639/denosumab-for-the-treatment-of-adult-multisystem-langerhans-cell-histiocytosis
#11
Polyzois Makras, Marina Tsoli, Athanasios D Anastasilakis, Maria Thanou, Gregory Kaltsas
PURPOSE: An etiological treatment is currently lacking for Langerhans Cell Histiocytosis (LCH). Receptor activator of nuclear factor κB ligand (RANKL) appears to play a central role in the lesional immunological process inducing compensatory osteoprotegerin (OPG) activation. In a preliminary study we aimed to evaluate for the first time the use of denosumab, a RANKL inhibitor, as a targeted treatment strategy in LCH in order to support and enhance endogenous OPG action in order to control or alter the lesional immunological process...
April 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28274065/multifocal-eosinophilic-granuloma-of-jaws-and-skull-with-classical-and-unusual-radiographic-imaging-findings
#12
Jayam Raviraj, Suman Venkata, Sameulla Shaik, Amrutha Kodadala, Prashanti Kakarla
Eosinophilic granuloma is basically a disorder of reticuloendothelial system and is one of the variants of langerhans cell histiocytosis. Multifocal eosinophilic granuloma affecting jaws and skull is relatively a rare disorder. We hereby report a case of multifocal eosinophilic granuloma involving mandible, maxilla and several skull bones. The present case has mixture of classical floating teeth appearance and an unusual radiographic/imaging finding of periosteal remodeling, which is rarely seen in adult patients of eosinophilic granuloma and pseudo-multilocular appearance in anterior mandibular region in coronal sections and moth-eaten appearance of skull was appreciated in axial slices of Computed Tomography (CT)...
January 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28268124/-usefulness-of-nuclear-medicine-in-erdheim-chester-disease-a-lille-experience
#13
A Adens, P Landy, L Terriou, C Baillet, A Beron, M Lambert, D Launay, D Huglo
INTRODUCTION: Erdheim-Chester disease is a rare form of non-langerhans histiocytosis and its etiology is still not well established. The aims of the study were to assess the value of the bone scintigraphy and the (18)F-FDG PET/CT for the diagnostic and for the latter in the therapeutic evaluation. METHODS: We retrospectively reviewed 49 patients suspected of Erdheim-Chester disease between 2004 and 2016. Bone scintigraphy was compared with histopathology and PET-CT to conventional morphological examinations and bone scintigraphy...
March 3, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28264540/diagnosis-and-treatment-of-cystic-lung-disease
#14
REVIEW
Sanghoon Park, Eun Joo Lee
Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually < 2 mm). The recognition of CLDs has increased with the widespread use of computed tomography. This article addresses the mechanisms of cyst formation and the diagnostic approaches to CLDs. A number of assessment methods that can be used to confirm CLDs are discussed, including high-resolution computed tomography, pathologic approaches, and genetic/ serologic markers, together with treatment modalities, including new therapeutic drugs currently being evaluated...
March 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28262495/-18-f-fdg-pet-ct-in-follow-up-evaluation-in-pediatric-patients-with-langerhans-histiocytosis
#15
J R Garcia, E Riera, P Bassa, S Mourelo, M Soler
PURPOSE: We evaluated the impact of (18)F-FDG PET/CT in identifying sites of active disease and to assess therapeutic follow up in a group of pediatric patients with Langerhans cell histiocytosis (LCH). METHOD: During 2007-2013, 13 (18)F-FDG PET/CT studies were performed for follow-up in 7 patients with a diagnosis of LCH (4 female, 3 male; 1-12 years-old). PET findings were analyzed and correlated with the CT and MRI. Findings were also follow-up by these techniques...
March 2, 2017: Revista Española de Medicina Nuclear e Imagen Molecular
https://www.readbyqxmd.com/read/28255140/idiopathic-thrombocytopenic-purpura-in-childhood-langerhans-cell-histiocytosis-in-adulthood-more-than-a-chance-association
#16
Y C Kuan, M N Nurain
Described herein, a case of Langerhans cell histiocytosis (LCH) in an adult with Idiopathic Thrombocytopenic Purpura (ITP) diagnosed at age ten. She presented with cranial diabetes insipidus, later developed hypogonadotrophic hypogonadism and multiple cervical lympadenopathy from which histopathology of excisional biopsy confirmed LCH. Magnetic resonance imaging showed thickened pituitary stalk. Association of ITP and LCH is unknown but the question of LCH presenting as isolated thrombocytopenia in childhood only to be discovered in adulthood when there was pituitary and bone involvement remains...
February 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28248858/severe-sclerosing-cholangitis-after-langerhans-cell-histiocytosis-treated-by-liver-transplantation-an-adult-case-report
#17
Yunhua Tang, Zhiheng Zhang, Maogen Chen, Weiqiang Ju, Dongping Wang, Fei Ji, Qingqi Ren, Zhiyong Guo, Xiaoshun He
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder of unknown pathogenesis. LCH diseases may occur in a single organ or multisystem organ. The patients with multisystem involvement usually have a poor prognosis. Liver involvement in multisystem LCH results in severe complications, such as obvious sclerosing cholangitis (SC) with jaundice. METHODS: We reported a 31-year-old man developed severe SC due to multisystem LCH and was successfully treated by liver transplantation (LT)...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28247189/successful-treatment-of-histiocytic-sarcoma-with-cladribine-and-high-dose-cytosine-arabinoside-in-a-child
#18
Haruko Iwabuchi, Hiroyuki Kawashima, Hajime Umezu, Takayuki Takachi, Masaru Imamura, Akihiko Saitoh, Akira Ogose, Chihaya Imai
Histiocytic sarcoma, a rare hematopoietic neoplasm with evidence of histiocytic differentiation, is often refractory to conventional chemotherapy and radiotherapy, and its prognosis is generally dismal. The optimal management of this malignancy has not been established. We report a case of 8-year-old girl with histiocytic sarcoma involving the left femur. The tumor rapidly responded to a combination of cladribine and high-dose cytosine arabinoside, an aggressive salvage regimen for refractory Langerhans cell histiocytosis, and became impalpable during the first cycle...
February 28, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28225310/central-diabetes-insipidus-clinical-characteristics-and-long-term-course-in-a-large-cohort-of-adults
#19
Hiba Masri-Iraqi, Dania Hirsch, Dana Herzberg, Avner Lifshitz, Gloria Tsvetov, Carlos Benbassat, Ilan Shimon
Purpose Central diabetes insipidus (CDI) is a rare heterogeneous condition with various underlying causes. This study sought to increase the still-limited data on the clinical characteristics and long-term course in adults diagnosed with CDI. Methods Data on demographics, presentation, imaging findings, affected pituitary axes, treatment, and complications were collected retrospectively from the files of 70 adult patients with CDI followed at a referral endocrine clinic. Results 40 women and 30 men were included...
February 22, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28220299/molecular-analysis-of-braf-v600e-mutation-in-multiple-nodules-of-pulmonary-langerhans-cell-histiocytosis
#20
Arno Dimmler, Helene Geddert, Martin Werner, Gerhard Faller
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related histiocytic disorder with variable clinical symptoms. Like in other non-pulmonary Langerhans cell proliferations, PLCH has recently been shown to harbour BRAF V600E mutations in a significant subset of cases, thus challenging the concept of PLCH being a reactive disorder. Here, we analysed 38 formalin-fixed and paraffin-embedded PLCH nodules of nine patients for BRAF mutation using two different molecular methods. Using pyrosequencing and allele-specific quantitative PCR (AS-PCR), BRAF V600E mutations were found in 16/38 (42%) and 31/37 (84%) nodules, respectively...
February 20, 2017: Virchows Archiv: An International Journal of Pathology
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