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Langerhans histiocytosis

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https://www.readbyqxmd.com/read/29774135/vemurafenib-in-langerhans-cell-histiocytosis-report-of-a-pediatric-patient-and-review-of-the-literature
#1
Anne Heisig, Jan Sörensen, Stefanie-Yvonne Zimmermann, Stefan Schöning, Dirk Schwabe, Hans-Michael Kvasnicka, Raphaela Schwentner, Caroline Hutter, Thomas Lehrnbecher
Selective BRAF inhibitors such as vemurafenib have become a treatment option in patients with Langerhans cell Histiocytosis (LCH). To date, only 14 patients receiving vemurafenib for LCH have been reported. Although vemurafenib can stabilize the clinical condition of these patients, it does not seem to cure the patients, and it is unknown, when and how to stop vemurafenib treatment. We present a girl with severe multisystem LCH who responded only to vemurafenib. After 8 months of treatment, vemurafenib was tapered and replaced by prednisone and vinblastine, a strategy which has not been described to date...
April 24, 2018: Oncotarget
https://www.readbyqxmd.com/read/29768711/clinical-resistance-associated-with-a-novel-map2k1-mutation-in-a-patient-with-langerhans-cell-histiocytosis
#2
David O Azorsa, David W Lee, Daniel H Wai, Ranjan Bista, Apurvi R Patel, Eiman Aleem, Michael M Henry, Robert J Arceci
Patients with Langerhans cell histiocytosis (LCH) harbor BRAF V600E and activating mutations of MAP2K1/MEK1 in 50% and 25% of cases, respectively. We evaluated a patient with treatment-refractory LCH for mutations in the RAS-RAF-MEK-ERK pathway and identified a novel mutation in the MAP2K1 gene resulting in a p.L98_K104 > Q deletion and predicted to be auto-activating. During treatment with the MEK inhibitor trametinib, the patient's disease showed significant progression. In vitro characterization of the MAP2K1 p...
May 16, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29765943/juvenile-xanthogranuloma-in-a-pediatric-patient-with-langerhans-cell-histiocytosis
#3
Radha Ram, Marissa D Marolf, Patricia Chévez-Barrios, Honey H Herce
Purpose: To report a case of juvenile xanthogranuloma that simulated a chalazion and to discuss the association between juvenile xanthogranuloma and Langerhans cell histiocytosis. Method: Case report and review of literature. Results: A 13-year-old boy with a prior history of Langerhans cell histiocytosis was referred to our clinic for a possible chalazion. The patient had undergone treatment for Langerhans cell histiocytosis 10 years prior...
April 2018: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/29755272/langerhans-cell-histiocytosis-in-an-18-month-old-child-presenting-as-periorbital-cellulitis
#4
Andre Ali-Ridha, Seymour Brownstein, Michael O'Connor, Steve Gilberg, Tina Tang
Langerhans cell histiocytosis (LCH) is a rare multi-system disease. It presents infrequently as a childhood orbital tumor, and can mimic more common inflammatory orbital disease processes. We report the clinical, histopathological, and electron microscopic findings of orbital LCH in an 18-month-old child, along with a review of the recent literature regarding molecular pathogenetic analysis of LCH. The child presented with a two-week history of progressive left periorbital edema and redness. He was initially diagnosed and treated empirically for bacterial periorbital cellulitis, but subsequently underwent ophthalmological consultation after he failed to improve...
January 2018: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/29754886/langerhans-cell-histiocytosis-in-children-diagnosis-differential-diagnosis-treatment-sequelae-and-standardized-follow-up
#5
REVIEW
Jolie Krooks, Milen Minkov, Angela G Weatherall
A definitive diagnosis of Langerhans cell histiocytosis (LCH) requires a combination of clinical presentation, histology, and immunohistochemistry. The inflammatory infiltrate contains various proportions of LCH cells, the disease hallmark, which are round and have characteristic "coffee-bean" cleaved nuclei and eosinophilic cytoplasm. Positive immunohistochemistry staining for CD1a and CD207 (langerin) are required for a definitive diagnosis. Isolated cutaneous disease should only be treated when symptomatic, because spontaneous resolution is common...
June 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29754885/langerhans-cell-histiocytosis-in-children-history-classification-pathobiology-clinical-manifestations-and-prognosis
#6
REVIEW
Jolie Krooks, Milen Minkov, Angela G Weatherall
Langerhans cell histiocytosis (LCH) is an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase pathway. When disease involves the skin, LCH most commonly presents as a seborrheic dermatitis or eczematous eruption on the scalp and trunk. Evaluation for involvement of other organ systems is essential, because 9 of 10 patients presenting with cutaneous disease also have multisystem involvement. Clinical manifestations range from isolated disease with spontaneous resolution to life-threatening multisystem disease...
June 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29743654/expression-of-the-transcription-factor-zbtb46-distinguishes-human-histiocytic-disorders-of-classical-dendritic-cell-origin
#7
Ansuman T Satpathy, Ryanne A Brown, Ellen Gomulia, Carlos G Briseño, Maxwell R Mumbach, Zenggang Pan, Kenneth M Murphy, Yasodha Natkunam, Howard Y Chang, Jinah Kim
Distinguishing classical dendritic cells from other myeloid cell types is complicated by the shared expression of cell surface markers. ZBTB46 is a zinc finger and BTB domain-containing transcription factor, which is expressed by dendritic cells and committed dendritic cell precursors, but not by plasmacytoid dendritic cells, monocytes, macrophages, or other immune cell populations. In this study, we demonstrate that expression of ZBTB46 identifies human dendritic cell neoplasms. We examined ZBTB46 expression in a range of benign and malignant histiocytic disorders and found that ZBTB46 is able to clearly define the dendritic cell identity of many previously unclassified histiocytic disease subtypes...
May 9, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29737419/langerhans-cell-histiocytosis-presenting-as-crohn-s-disease-a-case-report
#8
Amelie Therrien, Zaki El Haffaf, Claire Wartelle-Bladou, Justin Côté-Daigneault, Bich N Nguyen
PURPOSE: We describe an exceptional case of Langerhans cell histiocytosis (LCH) that presented as Crohn's disease and primary sclerosing cholangitis. METHODS: The patient's clinical, endoscopic, and histologic data from the Centre Hospitalier de l'Universite de Montreal were reviewed, as well as the literature on LCH involving the digestive tract and the liver, with a focus on the similarities with Crohn's disease and primary sclerosing cholangitis. RESULTS: A 39 years-old man first presented with anal fissures and deep punctiform colonic ulcers...
May 8, 2018: International Journal of Colorectal Disease
https://www.readbyqxmd.com/read/29729245/at-the-root-cutaneous-langerhans-cell-histiocytosis-%C3%A2-%C3%A2-%C3%A2-%C3%A2-%C3%A2-%C3%A2-%C3%A2
#9
Roberto Maglie, Margherita Vannucchi, Lavinia Quintarelli, Marzia Caproni, Daniela Massi, Emiliano Antiga
No abstract text is available yet for this article.
May 2, 2018: American Journal of Medicine
https://www.readbyqxmd.com/read/29724524/the-role-of-parental-and-perinatal-characteristics-on-langerhans-cell-histiocytosis-characterizing-increased-risk-among-hispanics
#10
Erin C Peckham-Gregory, Kenneth L McClain, Carl E Allen, Michael E Scheurer, Philip J Lupo
PURPOSE: Potential roles of inherited and environmental risk factors in pathogenesis of Langerhans cell histiocytosis (LCH), a myeloid neoplastic disorder, are undefined. We therefore evaluated the role of parental and perinatal factors on the risk of this childhood cancer. METHODS: Information on LCH cases (n = 162) for the period 1995-2011 was obtained from the Texas Cancer Registry. Birth certificate controls were frequency-matched on year of birth at a ratio of 10:1 for the same period...
April 17, 2018: Annals of Epidemiology
https://www.readbyqxmd.com/read/29720485/consensus-recommendations-for-the-diagnosis-and-clinical-management-of-rosai-dorfman-destombes-disease
#11
Oussama Abla, Eric Jacobsen, Jennifer Picarsic, Zdenka Krenova, Ronald Jaffe, Jean-Francois Emile, Benjamin H Durham, Jorge Braier, Frédéric Charlotte, Jean Donadieu, Fleur Cohen Aubart, Carlos Rodriguez-Galindo, Carl Allen, James A Whitlock, Sheila Weitzman, Kenneth L McClain, Julien Haroche, Eli L Diamond
Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by accumulation of activated histiocytes within affected tissues. RDD, which now belongs to the R group of the 2016 revised histiocytoses classification, is a widely heterogeneous entity with a range of clinical phenotypes occurring in isolation or in association with autoimmune or malignant diseases. Recent studies have found NRAS, KRAS, MAP2K1 and ARAF mutations in lesional tissues, raising the possibility of a clonal origin in some forms of RDD...
May 2, 2018: Blood
https://www.readbyqxmd.com/read/29714464/insulin-like-growth-factor-1-receptor-expression-in-pediatric-tumors-a-comparative-immunohistochemical-study
#12
Resul Karakuş, Esra Karakuş, Suna Emir, Ayper Kaçar, Derya Özyörük
Background/aim: Insulin-like growth factor-1 receptor (IGF-1R) is a pivotal receptor tyrosine kinase involved in the cell cycle and malignant tumor transformation. It is differentially expressed in various types of tumors. We aimed to determine the expression of IGF- 1R in different pediatric tumors and to shed light on possible new indications of anti-IGF-1R treatment approaches. Materials and methods: A total of 147 specimens were analyzed according to their expression of IGF-1R. Specimens included those from rhabdomyosarcomas, Wilms tumors, Ewing sarcoma/primitive neuroectodermal tumors, peripheral neuroblastic tumors, acute lymphoblastic lymphoma, Hodgkin lymphoma, Burkitt lymphoma, retinoblastoma, pleuropulmonary blastoma, Langerhans cell histiocytosis, endodermal sinus tumors (ESTs), and myeloid sarcoma...
April 30, 2018: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29713572/langerhans-cell-histiocytosis-of-the-gastrointestinal-tract-a-rare-entity
#13
Jasmine Bhinder, Amit Mori, Leon Kurtz, Madhavi Reddy
Langerhans cell histiocytosis (LCH) is an idiopathic and rare disease that ranges in clinical severity based on location and organ involvement. LCH most commonly affects the skin and bones. The involvement of the gastrointestinal tract (GI) in adults is exceedingly rare and only 10 cases have been reported in the literature. We present the case of a 60-year-old male who was referred for a routine screening colonoscopy. Numerous 3-5 mm nodular lesions were present throughout the colon. A histopathological examination revealed diffuse aggregates of histiocytes within the lamina propria of the mucosa and immunohistochemical staining further confirmed the presence of Langerhans cells with a positive CD1-a stain...
February 26, 2018: Curēus
https://www.readbyqxmd.com/read/29708356/mapk-erk-signal-pathway-alterations-in-patients-with-langerhans-cell-histiocytosis
#14
Olga Novosad, Tanya Skrypets, Yan Pastushenko, Iryna Titorenko, Arina Martynchyk, Oksana Skachkova, Mariia Inomistova, Alex Gorbach, Nataliia Khranovska, Irina Kryachok
BACKGROUND: Clinical outcomes of Langerhans cell histiocytosis (LCH) are highly variable. It has been suggested that mitogen-activated protein kinase (MAPK) /extracellular signal-regulated kinases (ERK) signaling pathway might be activated in LCH patients. MATERIALS AND METHODS: We investigated KRAS, BRAF and NRAS mutations in patients with LCH by qPCR. RESULTS: Eight adult patients with LCH were treated at the National Cancer Institute, Kiev, Ukraine...
2018: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/29702989/the-application-of-x-ray-computed-tomography-and-magnetic-resonance-imaging-on-22-pediatric-langerhans-cell-histiocytosis-patients-with-long-bone-involvement-a-retrospective-analysis
#15
Xiaojun Zhang, Jing Zhou, Xuee Chai, Guiling Chen, Bin Guo, Lei Ni, Peng Wu
The studies focusing on x-ray, computed tomography (CT), and magnetic resonance imaging (MRI) in pediatric Langerhans cell histiocytosis (LCH) patients were still rare. Therefore, we aimed to evaluate the application of x-ray, CT, and MRI in pediatric LCH patients with long bone involvement.Total 22 pediatric LCH patients were included in this study. The diagnosis of LCH was confirmed by pathological examination. All patients were followed up for 3 years. X-ray, CT, or MRI was performed and the results were recorded for further analyses...
April 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29694792/response-to-trametinib-of-a-pulmonary-langerhans-cell-histiocytosis-harboring-a-map2k1-deletion
#16
Gwenaël Lorillon, Fanelie Jouenne, Barouyr Baroudjian, Constance de Margerie-Mellon, Laetitia Vercellino, Véronique Meignin, Celeste Lebbe, Robert Vassallo, Samia Mourah, Abdellatif Tazi
No abstract text is available yet for this article.
April 25, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29693798/treating-langerhans-cell-histiocytosis-globally
#17
Stephan Ladisch
No abstract text is available yet for this article.
April 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29691886/cutaneous-langerhans-cell-histiocytosis-presenting-with-hypopigmented-lesions-report-of-two-cases-and-review-of-literature
#18
Shoko Mori, Tony Adar, Viktoryia Kazlouskaya, Jaime B Alexander, Edward Heilman, Sharon A Glick
Langerhans cell histiocytosis is a rare group of disorders that results from the abnormal proliferation and accumulation of dendritic-derived cells in various organs of the body, such as the skin and bones. Hypopigmented macules are a rare cutaneous presentation of Langerhans cell histiocytosis that may pose a diagnostic dilemma when no other findings of Langerhans cell histiocytosis are present at the time of examination. We present 2 cases of the hypopigmented variant of Langerhans cell histiocytosis, including a case with histopathologic features of regression, and a review of the literature...
April 25, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29675756/nodular-macroregenerative-tissue-as-a-pattern-of-regeneration-in-cholangiopathic-disorders
#19
Preston Roberts, Andrew T Trout, Jonathan R Dillman
BACKGROUND: Published case series have described central hepatic macroregenerative nodules or masses as a common feature of Alagille syndrome. Our experience suggests this regenerative pattern can be seen more generally in cholangiopathic disorders. OBJECTIVE: To define the frequency of central regenerative tissue in Alagille syndrome and other cholangiopathic disorders and to describe the typical appearance of such regenerative tissue. MATERIALS AND METHODS: We conducted a retrospective study of CT and MR imaging performed in children and young adults with cholangiopathic disorders between January 2000 and June 2016...
April 19, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29658453/-braf-v600e-mutation-and-its-clinical-significance-in-children-with-langerhans-cell-histiocytosis
#20
Xue Tang, Xia Guo, Lin-Yong Sun, Yuan Ai, Xue Yang, Jing-Jing Sun, Jian-Rong Wu, Ju Gao
OBJECTIVE: To investigate the clinical significance of BRAF-V600E mutation in children with Langerhans cell histiocytosis (LCH). METHODS: Real-time fluorescence quantitative PCR was used to detect BRAF-V600E mutation in paraffin-embedded tissue samples from 26 children with LCH. A retrospective analysis was performed for the association of BRAF-V600E mutation with clinical features and prognosis of children with LCH. RESULTS: Of the 26 children, 25 received standard chemotherapy, with a 2-year overall survival (OS) rate of 100% and a 2-year event-free survival (EFS) rate of 88%...
April 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
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