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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
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
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
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
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
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
Edzard Hangen, Stefan Gonser, Jutta Köhler
Polychlorinated dibenzo-p-dioxins and dibenzo-furans (PCDD/Fs) belong to the most toxic persistent organic environmental pollutants. Therefore, knowledge about their fate along the exposure path is of special concern. In order to comprehend the transfer of PCDD/Fs across different environmental compartments, PCDD/F concentrations in plants and in soil were evaluated. Pairs of soil and plant samples were selected according to the shortest distance between sampling points. At 15 sites PCDD/F concentrations in spruce needles and in forest humus layers where compared...
August 1, 2018: Science of the Total Environment
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
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
Joseph R Pauszek, Bradley S Gibson
Here we propose a rational analysis account of voluntary symbolic attention control-the Least Costs Hypothesis (LCH)-that construes voluntary control as a decision between intentional cue use and unguided search. Consistent with the LCH, the present study showed that this decision is sensitive to variations in cue processing efficiency. In Experiment 1, observers demonstrated a robust preference for using "easy-to-process" arrow cues but not "hard-to-process" spatial word cues to satisfy an easy visual search goal; Experiment 2 showed that this preference persisted even when the temporal costs of cue processing were neutralized...
April 30, 2018: Journal of Experimental Psychology. Human Perception and Performance
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
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)
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
Hui Yan Ong, Liang Chye Goh, Kalimuthu Santhi, Mohamad Mokhtar Sha'ariyah
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder, which commonly arises in the bone and may involve other systems. To date, the diagnosis of temporal bone LCH remains a challenge as it may masquerade as a common ear infection. We report a case of a child who presented to us with persistent bilateral ear discharge for four months and was not responding to treatment. Her condition subsequently worsened, with clinical features and radiological findings suggestive of mastoid cellulitis. Nevertheless, further histopathology study revealed LCH...
March 2018: Oman Medical Journal
Lisa M McGinnis, Grant Nybakken, Lisa Ma, Daniel A Arber
Langerhans cell histiocytosis is a proliferative disorder of neoplastic Langerhans cells with activating mutations in the Erk signaling pathway. TP53 and U2AF1 mutations have been implicated in other myelomonocytic malignancies and we hypothesized that mutations in these genes may cosegregate in LCH patients according to BRAF mutation status. Towards this end, we collected cases with a pathologic diagnosis of Langerhans cell histiocytosis from Stanford University Hospital. We analyzed the status of known pathogenic alleles in BRAF, ARAF, TP53, U2AF1, and MAP2K1 on formalin-fixed, paraffin-embedded tissue by direct sequencing...
April 11, 2018: American Journal of Surgical Pathology
Celeste Sánchez-Romero, Ana Isabel Cuenca Arriaga, Oslei Paes de Almeida, Evangelina Gutiérrez Cortés
Juvenile Xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis (non-LCH) affecting normolipemic infants and children most frequently in the first year of life, often showing spontaneous regression within 3 to 6 years. Classic JXG is characterized by a yellowish asymptomatic papule or nodule, often located in the skin of the head, neck and upper trunk. Oral JXG have been reported, but is rare. Histologically JXG is composed mainly by an infiltrate of macrophages with a variable degree of lipidization (foamy macrophages), and (most of the times) scattered Touton type giant cells...
April 6, 2018: Journal of Cutaneous Pathology
Kenneth L McClain, Jennifer Picarsic, Rikhia Chakraborty, Daniel Zinn, Howard Lin, Harshal Abhyankar, Brooks Scull, Albert Shih, Karen Phaik Har Lim, Olive Eckstein, Joseph Lubega, Tricia L Peters, Walter Olea, Thomas Burke, Nabil Ahmed, M John Hicks, Brandon Tran, Jeremy Jones, Robert Dauser, Michael Jeng, Robert Baiocchi, Deborah Schiff, Stanton Goldman, Kenneth M Heym, Harry Wilson, Benjamin Carcamo, Ashish Kumar, Carlos Rodriguez-Galindo, Nicholas S Whipple, Patrick Campbell, Geoffrey Murdoch, Julia Kofler, Simon Heales, Marian Malone, Randy Woltjer, Joseph F Quinn, Paul Orchard, Michael C Kruer, Ronald Jaffe, Markus G Manz, Sergio A Lira, D Williams Parsons, Miriam Merad, Tsz-Kwong Man, Carl E Allen
BACKGROUND: Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH. METHODS: Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions...
April 6, 2018: Cancer
Ranju Kharel Sitaula, Anadi Khatri
Bilateral intraocular involvement in Langerhans cell histiocytosis (LCH) is uncommon. A 15-year-old boy presented with painless decreased vision in right and painful left red eye of 2 weeks duration. Visual acuity was 20/500 and 20/200, respectively. A fixed dilated pupil with exudative retinal detachment was present in the right eye and hemorrhagic iris nodules with hyphema and hypopyon were seen in the left eye. Intraocular pressure was 12 and 31 mmHg, respectively, in each eye. Ocular symptoms were preceded by fever with multiple skin rashes, subcutaneous nodules, and lymph node enlargement...
2018: International Medical Case Reports Journal
Akira Morimoto, Yoko Shioda, Toshihiko Imamura, Kazuko Kudo, Toshiyuki Kitoh, Hiroshi Kawaguchi, Hiroaki Goto, Yoshiyuki Kosaka, Yukiko Tsunematsu, Shinsaku Imashuku
Langerhans cell histiocytosis (LCH) with single-system (SS) multifocal bone (MFB) lesions is rarely fatal, but patients may experience relapses and develop LCH-associated sequelae. To evaluate effect on outcomes of pediatric multifocal LCH, we tested a treatment protocol modified from the Japan Langerhans Cell Histiocytosis Study Group (JLSG)-96 study. We assessed the outcomes of all consecutive newly diagnosed pediatric patients with LCH with SS-MFB lesions who were treated with JLSG-02 protocol in 2002-2009...
March 28, 2018: International Journal of Hematology
Hui Zheng, Zhengrong Xia, Wenjun Cao, Yun Feng, Shuxian Chen, Yu-Hua Li, Deng-Bin Wang
BACKGROUND: We aimed to evaluate the clinical and imaging presentations of Langerhans cell histiocytosis (LCH) in the pediatric temporal bone. METHODS: This retrospective study included 27 pediatric cases with pathological confirmed LCH of the temporal bone. The clinical and imaging features of the cases were analyzed. The involvement of ossicular chain and otic capsule was also evaluated. RESULTS: A total of 38 lesions (27 cases) with 11 bilateral involvement were identified...
March 27, 2018: World Journal of Surgical Oncology
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