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Langerhans cell histiocytosis

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https://www.readbyqxmd.com/read/28903440/evaluation-of-pituitary-uptake-incidentally-identified-on-18-f-fdg-pet-ct-scan
#1
Huijun Ju, Jinxin Zhou, Yu Pan, Jing Lv, Yifan Zhang
The clinical significance of pituitary uptake on routine whole body (18)F-fluorodeoxyglucose (FDG) positron emission tomography/computer tomography (PET/CT) is not completely characterized. We seek to assess the potential differential diagnosis/underlying etiology of pituitary FDG uptake incidentally identified on routine PET/CT scans. A total of 24,007 PET/CT whole body scans in recent 5 years were retrospectively reviewed. Patients with maximum standardized uptake value (SUVmax) > 4.1 in the pituitary glands were identified...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28880370/langerhans-cell-histiocytosis-in-an-adult-female-presenting-with-widespread-confluent-crusted-papules-and-review-of-adult-cases-confined-to-skin
#2
Shiyu Zhang, Yuehua Liu
No abstract text is available yet for this article.
September 7, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28877978/how-i-manage-pulmonary-langerhans-cell-histiocytosis
#3
Gwenaël Lorillon, Abdellatif Tazi
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare sporadic cystic lung disease of unknown aetiology that is characterised by the infiltration and destruction of the wall of distal bronchioles by CD1a(+) Langerhans-like cells. In adults, PLCH is frequently isolated and affects young smokers of both sexes. Recent multicentre studies have led to the more standardised management of patients in clinical practice. Smoking cessation is essential and is occasionally the only suitable intervention. Serial lung function testing is important because a significant proportion of patients may experience an early decline in forced expiratory volume in 1 s and develop airflow obstruction...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28868307/atlanto-axial-langerhans-cell-histiocytosis-in-a-child-presented-as-torticollis
#4
Miniar Tfifha, Mehdi Gaha, Nadia Mama, Mohamed Taher Yacoubi, Saoussen Abroug, Hela Jemni
Langerhans cell histiocytosis (LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma (EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many reports of patients with spine histiocytosis are well documented in the literature but it is not the case of atlantoaxial localization. We report here a new observation of atlantoaxial LCH in a 4-year-old boy revealed by persistent torticollis. He was successfully treated with systemic chemotherapy and surgery...
August 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28868020/erdheim-chester-disease-the-importance-of-information-integration
#5
Anna Nikonova, Khashayar Esfahani, Guillaume Chausse, Stephan Probst, Tina Petrogiannis-Haliotis, Hans Knecht, Genevieve Gyger
BACKGROUND: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. CASE PRESENTATION: We describe herein a patient with a history of stroke-like symptoms and retroperitoneal fibrosis that on initial pathology raised the possibility of IgG4-related disease...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28864216/langerhans-cell-histiocytosis-mimicking-periapical-pathology-in-a-39-year-old-man
#6
Scott M Peters, Julie Pastagia, Angela J Yoon, Elizabeth M Philipone
Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type dendritic cells, with more than 50% of cases of LCH seen in children younger than 15 years of age. The most common clinical presentation of LCH is solitary or multiple bony lesions. The jaws are affected in approximately 10%-20% of cases, with a strong predilection for the mandible. The maxilla is involved in only 1% of head and neck cases. When the jaws are involved, lesions of LCH may mimic periapical pathology as seen in patients requiring endodontic therapy or bone loss as seen in periodontal disease...
August 29, 2017: Journal of Endodontics
https://www.readbyqxmd.com/read/28859038/refractory-lch-with-secondary-intracranial-pnet-a-case-report-and-review-of-literature
#7
Varsha Chayapathi, Amita Mahajan, Manas Kalra
The occurrence of second malignant neoplasms in patients with Langerhans cell histiocytosis is infrequent but has been reported. Here we report the case of a child with refractory Langerhans cell histiocytosis who was treated with cladribine and later developed a secondary intracranial primitive neuroectodermal tumor. The possible association of cladribine with second neoplasm is further discussed.
August 30, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28858125/langerhans-cell-histiocytosis-of-the-thyroid-complicated-by-papillary-thyroid-carcinoma-a-case-report-and-brief-literature-review
#8
REVIEW
Xin Wu, Shi Chen, Li-Yang Zhang, Ya-Ping Luo, Ying Jiang, Rui-E Feng
RATIONALE: Langerhans cell histiocytosis (LCH) involves mainly the skin and bone and rarely the thyroid. Meanwhile, papillary thyroid carcinoma (PTC) is the most common subtype of thyroid cancer. Both LCH and PTC could make the thyroid enlarged and hypermetabolic. The coincidence of these 2 events in a patient is rare, and this paper aimed to report such case. PATIENT CONCERNS: A 40-year-old man presented with polyuria and polydipsia for 5 years. The symptoms had been relieved well by drug therapy for >4 years, until the drugs could not control the symptoms anymore and an extensively enlarged thyroid gland was noticed...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28836893/systemic-juvenile-xanthogranuloma-involving-the-bone-marrow-multiple-bones-and-the-skin-that-developed-during-treatment-of-acute-lymphoblastic-leukemia-in-remission-state
#9
Eunjae Cheon, Saemi Yang, Jae Ho Han, Kwang Chul Lee, Jun Eun Park
Juvenile xanthogranuloma (JXG) is a rare benign disorder classified as non-Langerhans cell histiocytosis, with unclear etiology and pathogenesis. JXG is generally characterized by solitary or multiple cutaneous nodules that resolve spontaneously over a few years. JXG rarely presents as extracutaneous lesions that progress to a symptomatic systemic disorder through multiple organ involvement. We encountered a systemic JXG case involving the bone marrow, multiple bones, and the skin during acute lymphoblastic leukemia treatment...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28834636/intraosseous-rosai-dorfman-disease-diagnosed-by-touch-imprint-cytology-evaluation-a-case-series
#10
Avani A Pendse, Sara E Wobker, Kevin G Greene, Scott V Smith, Robert J Esther, Leslie G Dodd
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD) is a rare benign disorder that primarily affects the lymph nodes. Localized lymphadenopathy is the most common clinical manifestation of this disorder. However, RDD has been described in several extra-nodal sites including the head and neck region, soft tissue, skin, upper respiratory tract, gastro-intestinal tract and central nervous system (CNS). Involvement of the bone is considered very rare, occurring in less than 10% patients...
August 23, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28825606/cutaneous-indeterminate-cell-histiocytosis-of-donor-origin-after-allogeneic-hematopoietic-stem-cell-transplantation
#11
Angel Santos-Briz, Concepción Román, Rocío Corral, Alvaro de Dios, Lourdes Vázquez, María D Ludeña
Allogeneic hematopoietic stem-cell transplantation and solid-organ transplantation are associated with an increased risk of secondary neoplasms. Indeterminate cell histiocytosis (ICH) is a rare disease composed of so-called indeterminate cells, an alleged cutaneous dendritic cell subset displaying histological and some ultrastructural and immunophenotypic features of Langerhans cells but lacking Birbeck granules. We report a case of cutaneous ICH occurring after allogeneic hematopoietic stem-cell transplantation for a myelodysplastic syndrome in a 56-year-old man...
September 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28802389/cholesteatoma-as-a-complication-of-langerhans-cell-histiocytosis-of-the-temporal-bone-a-nationwide-cross-sectional-analysis
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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