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Langerhans histiocytosis

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https://www.readbyqxmd.com/read/28802389/cholesteatoma-as-a-complication-of-langerhans-cell-histiocytosis-of-the-temporal-bone-a-nationwide-cross-sectional-analysis
#1
Jonathan C Simmonds, Mark Vecchiotti
OBJECTIVE: To determine if patients with Langerhans Cell Histiocytosis (LCH) of the temporal bone have a higher risk of developing cholesteatoma. METHODS: Review of literature and cross-sectional weighted analysis of patients under 19 with a diagnosis of LCH from the National Inpatient Sample (NIS) and Kids' Inpatient Database (KID) from 2000 to 2013. ICD-9 codes and demographics were analyzed; pairwise comparisons and multivariate analyses were performed. RESULTS: Only seven cases of cholesteatoma after the treatment for LCH of the temporal bone have been documented in the literature...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28797508/hematopoietic-tumors-primarily-presenting-in-bone
#2
REVIEW
Arjen H G Cleven, Pancras C W Hogendoorn
Hematologic neoplasms that primarily present in bone are rare; this article describes the most common examples of hematologic tumors primarily presenting in bone, including plasma cell myeloma, solitary plasmacytoma of bone, primary non-Hodgkin lymphoma of bone, acute lymphoblastic leukemia/lymphoma, and Langerhans cell histiocytosis. The macroscopic and microscopic features, differential diagnosis, diagnostic workup, and prognosis of all these different entities are discussed, with special emphasis on common differential diagnosis...
September 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28794143/differential-diagnosis-of-granulomatous-lung-disease-clues-and-pitfalls-number-4-in-the-series-pathology-for-the-clinician-edited-by-peter-dorfm%C3%A3-ller-and-alberto-cavazza
#3
Shinichiro Ohshimo, Josune Guzman, Ulrich Costabel, Francesco Bonella
Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis)...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28791997/-eosinophilic-granuloma-of-the-parietal-bone-of-an-adult-patient-with-braf-mutation
#4
O V Dolzhansky, E M Paltseva, A A Bukaeva, E V Zaklyazminskaya, I A Spivak, D N Fedorov
The paper describes a case of eosinophilic granuloma of the parietal bone in a 32-year-old man. Histological examination revealed a large number of bean-shaped Langerhans cell histiocytes with lobed nuclei and nuclear grooves. The histiocytes alternated with the foci of obvious eosinophilic infiltration and with eosinophilic microabscesses. There were osteoclast-like multinucleated giant cells, bone resorption, and numerous bone rods covered with osteoblast chains. The histiocytes expressed CD1α, langerin, CD68, S100, and p53 (in 90...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28761697/cardiorespiratory-fitness-and-physical-function-in-children-with-cancer-from-diagnosis-throughout-treatment
#5
Troels Thorsteinsson, Hanne Baekgaard Larsen, Kjeld Schmiegelow, Lone Friis Thing, Peter Krustrup, Mogens Theisen Pedersen, Karl Bang Christensen, Pernille Rudebeck Mogensen, Anne Sofie Helms, Lars Bo Andersen
BACKGROUND: Children with cancer experience severe reductions in physical fitness and functionality during and following intensive treatment. This may negatively impact their quality of life. PURPOSE: To describe the physical capacity and functionality of children with cancer during and after treatment as well as the feasibility of physical activity intervention in the Rehabilitation including Social and Physical activity and Education in Children and Teenagers with Cancer study...
2017: BMJ Open Sport & Exercise Medicine
https://www.readbyqxmd.com/read/28759391/case-report-of-a-hypobaric-chamber-fitness-to-fly-test-in-a-child-with-severe-cystic-lung-disease
#6
Sarah Loo, Andrew Campbell, Julian Vyas, Naveen Pillarisetti
Patients with severe cystic lung disease are considered to be at risk for cyst rupture during air travel because of the possibility of increase in cyst size and impaired equilibration of pressure between the cysts and other parts of the lung. This may have clinically devastating consequences for the patient but may also result in significant costs for emergency alteration of flight schedule. We report the use of a hypobaric chamber to simulate cabin pressure changes encountered on a commercial flight to assess the safety to fly of a child with severe cystic lung disease secondary to Langerhans cell histiocytosis...
July 2017: Pediatrics
https://www.readbyqxmd.com/read/28757768/langerhans-cell-histiocytosis-of-the-bone
#7
Wen-Chih Huang, Ta-Pin Lee, Min-En Chou, Chien-Chen Tsai
No abstract text is available yet for this article.
January 2017: Ci Ji Yi Xue za Zhi, Tzu-chi Medical Journal
https://www.readbyqxmd.com/read/28748614/novel-activating-braf-fusion-identifies-a-recurrent-alternative-mechanism-for-erk-activation-in-pediatric-langerhans-cell-histiocytosis
#8
Sara Zarnegar, Benjamin H Durham, Pallavi Khattar, Neerav N Shukla, Ryma Benayed, Mario E Lacouture, Ehud Lavi, David C Lyden, Eli L Diamond, Ira J Dunkel, Omar Abdel-Wahab
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm characterized by constitutive activation of extracellular signal-regulated kinase (ERK). Genomic characterization has identified activating point mutations including mutually exclusive BRAFV600E and activating MAP2K1 mutations to be responsible for ERK activation in a majority of pediatric LCH patients. Here, we report the discovery of a novel BRAF kinase fusion, PACSIN2-BRAF, in a child with multisystem LCH. This is the second reported case of an activating BRAF kinase fusion and indicates a recurrent pathologic mechanism...
July 27, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28748503/role-of-18-f-fdg-pet-ct-in-patients-affected-by-langerhans-cell-histiocytosis
#9
Domenico Albano, Giovanni Bosio, Raffaele Giubbini, Francesco Bertagna
PURPOSE: Langerhans cell histiocytosis (LCH) is a rare hematological disorder for which the utility of(18)F-FDG PET/CT is unclear. Our aim was to explore the metabolic features of LCH and the possible role of(18)F-FDG PET/CT in LCH evaluation. MATERIALS AND METHODS: We found 17 patients with histologically proven LCH who underwent 17(18)F-FDG PET/CT scans for staging and 42 scans for restaging/follow-up purposes. PET/CT results were compared with those obtained from other conventional imaging modalities (bone scintigraphy, plain radiogram, computed tomography, magnetic resonance)...
July 26, 2017: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/28744781/lung-function-in-pregnancy-in-langerhans-cell-histiocytosis
#10
Elżbieta Radzikowska, Elżbieta Wiatr, Monika Franczuk, Iwona Bestry, Kazimierz Roszkowski-Śliż
Pulmonary Langerhans cell histiocytosis (LCH) is a rare disease, affecting usually young people. The course of the disease is variable. In some pulmonary LCH patients a severe lung destruction and progression in spite of chemotherapy is observed, but in others just a cessation of smoking induces a regression of the disease. In the present study we seek to determine the influence of pregnancy on pulmonary function in LCH patients, an unchartered area of research. We addressed the issue by investigating eight pregnant women out of the 45 women hospitalized with the diagnosis of pulmonary LCH in the period from 2000 to 2015...
July 26, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28742630/unique-case-of-hearing-recovery-after-otic-capsule-destruction-and-complete-sensorineural-hearing-loss-caused-by-langerhans-cell-histiocytosis
#11
Jennifer A Losie, Michael Yong, Frederick K Kozak, Neil K Chadha
: A 14-year-old woman presented with right-sided otologic and vestibular symptoms after presenting with hormonal disturbances earlier that year. Imaging showed a gross destruction of the temporal bone, mastoid air cells, and external acoustic meatus with invasion into the otic capsule. The patient experienced complete sensorineural hearing loss in the right ear. Biopsy diagnosed Langerhans cell histiocytosis (LCH) and the patient was treated with chemotherapy. After 1 year of treatment, the patient's hearing partially recovered and imaging showed reconstitution of the temporal bone including the otic capsule...
September 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28741604/bronchial-washing-cytology-of-pulmonary-langerhans-cell-histiocytosis-a-case-report
#12
Taeyeong Kim, Hyeong Ju Kwon, Minseob Eom, Sang Wook Kim, Min Hi Sin, Soon-Hee Jung
No abstract text is available yet for this article.
July 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28731671/-langerhans-cell-histiocytosis-presenting-as-isolated-adenitis-in-an-infant-case-report
#13
María Soriano-Ramos, Enrique Salcedo Lobato, María Baro Fernández, Daniel Blázquez-Gamero
Langerhans cell histiocytosis in infants is a rare condition, and presentation as an isolated cervical adenitis is exceptional at this age. We describe the case of a 3-month-old female infant presenting with a neck mass in the right mandibular angle with poor response to antibiotic treatment. Fine needle aspiration was performed and confirmed the diagnosis of Langerhans cell histiocytosis with complementary tests showing no features of systemic involvement. Langerhans cell histiocytosis should be considered in the differential diagnosis of subacute neck masses with poor outcome in infants and physicians should consider performing a fine needle aspiration to establish the diagnosis...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28731669/-langerhans-cell-histiocytosis-with-vertebral-involvement-and-soft-tissue-extension-clinical-case
#14
Tai C Luong, Adriana Scrigni, Marcela Paglia, Mariano Garavaglia, Nuria Aisenberg, Hernán Rowensztein, Claudia Sampor
Langerhans cell histiocytosis is a heterogeneous disease of unknown etiology characterized by proliferation of Langerhans cells. It is a rare disease. Bone involvement is common but vertebral disease is rare. We present a 4 year old patient with abdominal pain and neurologic symptoms. Magnetic resonance showed vertebra plana in D9 with involvement of paravertebral soft tissues. The child underwent surgery for decompression and biopsy. Biopsy confirmed Langerhans cell histiocytosis. She was treated with vinblastine and prednisone during 6 months following LCH-III with complete recovery of neurologic symptoms...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28730670/molluscum-contagiosum-like-presentation-of-langerhans-cell-histiocytosis-a-case-and-review
#15
Matthew S Karpman, Mohammed I AlJasser, Joseph M Lam
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation of Langerhans cells in the skin. A molluscum-like presentation of cutaneous LCH is rare but important to consider for examination and management. We present an atypical molluscum-like LCH case and review the literature for common features of this unusual presentation.
July 21, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28728220/-diagnostic-utility-of-tyrosine-hydroxylase-in-peripheral-neuroblastic-tumors
#16
N Zhang, N Liu, L J He
Objective: To investigation the diagnostic utility of tyrosine hydroxylase (TH) immunohistochemically as a marker of peripheral neuroblastic tumors(pNT). Methods: The study included 1 024 cases, 643 primary and metastatic pNT cases, 381 non-pNT cases, including small round cell tumors such as primitive neuroectodermal tumor (PNET), rhabdomyosarcoma, lymphoma, nephroblastoma, as well as other more common tumors (medulloblastoma, hepatoblastoma, pleuropulmonary blastoma, renal clear cell sarcoma, Langerhans cell histiocytosis, lipoblatoma etc)...
July 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28722363/-erdheim-chester-disease-a-differential-diagnosis-of-retroperitoneal-fibrosis
#17
Raphaël André, Jörg D Seebach
Erdheim-Chester disease is a rare multisystemic non-Langerhans histiocytosis with about 500 reported cases. Typical features include retroperitoneal and perirenal fibrosis (hairy kidney), periaortitis with a coated aorta, osteosclerosis of the lower limbs, and sometimes exophthalmia or diabetes insipidus. Histology is the cornerstone for diagnosis showing an infiltrate with foamy histiocytes and occasional multinucleated giant cells (Touton cells). There is no standard treatment regimen, current options include corticosteroids, interferon alpha, systemic chemotherapy, and radiation therapy ; however, a better understanding of the pathophysiological mechanisms has allowed the emergence of novel targeted treatments such as vemurafenib, imatinib, and anakinra...
April 5, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28711990/assessment-of-braf-v600e-mutation-in-pulmonary-langerhans-cell-histiocytosis-in-tissue-biopsies-and-bronchoalveolar-lavages-by-droplet-digital-polymerase-chain-reaction
#18
Clémence Pierry, Charline Caumont, Elodie Blanchard, Camille Brochet, Gael Dournes, Audrey Gros, Thomas Bandres, Séverine Verdon, Marion Marty, Hugues Bégueret, Jean-Philippe Merlio
The neoplastic nature of pulmonary Langerhans cell histiocytosis (PLCH) is still debated. As the detection of BRAF (V600E) and MAP2K1 mutations in patients with PCLH is now considered for such assessment, the aim of our study was to evaluate digital droplet polymerase chain reaction (ddPCR) in PCLH diagnosis. We retrospectively analyzed BRAF(V600E) detection in a cohort of 42 PCLH tissues and 18 bronchoalveolar lavages (BALs) by ddPCR, immunohistochemistry, high-resolution melting PCR (HRM), and next-generation sequencing (NGS)...
July 15, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28700967/eosinophilic-granuloma-at-the-cerebellopontine-angle-in-an-adult-a-rare-case-report-and-literature-review
#19
Arash Safarian, Nima Derakhshan, Mousa Taghipour, Amirreza Dehghanian
INTRODUCTION: Langerhans cell histiocytosis (LCH) is a rare immunologic disorder, identified by immature proliferation of histiocytes which may present as systemic or focal lesions. Eosinophilic granuloma (EG) is localized from of LCH mainly involving bones such as skull, femur, spine, ribs, mandible and pelvis. Cerebello-pontine (CP) angle is a rare anatomic location for involvement by EG. PRESENTATION OF CASE: A 32 year old man was being evaluated in our neuro-oncology clinic due to diplopia since 4 months ago...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28695297/histiocytic-and-dendritic-cell-neoplasms-what-have-we-learnt-by-studying-67-cases
#20
Fabio Facchetti, Stefano Aldo Pileri, Luisa Lorenzi, Valentina Tabanelli, Lisa Rimsza, Stefania Pittaluga, Stephan Dirnhofer, Christiane Copie-Bergman, Laurence de Leval, Andreas Rosenwald, Andrew Wotherspoon, Falko Fend
Tumors derived from histiocytic and dendritic cells encompass a large and heterogeneous group of neoplastic and reactive conditions, and their diagnosis is challenging both for pathologists and clinicians. Diagnosis is based on morphological and phenotypical findings, but hybrid features are not uncommon. Furthermore, recent studies uncovered the molecular mechanisms driving some of these tumors, improving diagnostic adequacy, and providing the basis for effective therapeutic breakthroughs.Sixty-seven cases were submitted to the accessory cell and histiocytic neoplasms session at the European Association of Haematopathology/Society for Hematopathology workshop 2016 held in Basel, Switzerland...
July 10, 2017: Virchows Archiv: An International Journal of Pathology
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