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Langerhans cell histiocytosis

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https://www.readbyqxmd.com/read/29125031/a-rapidly-expanding-calvarial-langerhans-cell-histiocytosis-with-low-ki-67-in-an-adult-a-challenging-diagnosis-on-magnetic-resonance-imaging
#1
Mustafa Kemal Demir, Ozlem Yapıcıer, Teyyub Hasanov, Deniz Kilic, Turker Kilic
Langerhans cell histiocytosis may have a wide variety of clinical presentations. The growth of localised form is usually slow and it mainly involves bones such as the skull, femur, spine, ribs, mandible and pelvis in children. The spectrum of clinical manifestations and magnetic resonance imaging findings of the disease may sometimes mimic infections as well as benign and malignant tumours. In this report, we describe an unusual case of rapidly progressive calvarial Langerhans cell histiocytosis with a painful and tender mass in a 27-year-old man that created diagnostic and therapeutic challenges...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/29120812/imaging-findings-in-systemic-childhood-diseases-presenting-with-dermatologic-manifestations
#2
REVIEW
Adam Z Fink, Julia K Gittler, Radhika N Nakrani, Jonathan Alis, Einat Blumfield, Terry L Levin
PURPOSE: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. METHODS: We review the imaging findings in childhood diseases associated with dermatologic manifestations. FINDINGS: Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura)...
October 31, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/29118704/spontaneous-remission-of-severe-systemic-langerhans-cell-histiocytosis-with-bladder-involvement-a-case-study
#3
Isotta M Magaton, Alexandar Tzankov, Fatime Krasniqi, Christof Rottenburger, Rosanna Zanetti-Daellenbach, Peter Grendelmeier, Viola Heinzelmann-Schwarz, Michael Mayr, Fabienne D Schwab
Background: The clinical presentation of Langerhans cell histiocytosis (LCH) is heterogeneous ranging from single-organ involvement to systemic disease causing substantial morbidity and mortality. We describe an unusual course of severe multisystem LCH with spontaneous remission. Case Presentation: We report on a 45-year-old Caucasian woman with cervical cancer, FIGO stage IVB. Five months after the end of combined radiochemotherapy and brachytherapy, the patient was readmitted because of severe dysuria...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29114286/intradural-eosinophilic-granuloma-invading-skull-case-report-and-review-of-the-literature
#4
Kavita Mardi, R C Thakur, Lalita Negi
Eosinophilic granuloma is a localized form of Langerhans cell histiocytosis, most commonly involving the skeletal system. Their origin from the dura is rare with only a handful of cases on record. We present one such rare case of an eosinophilic granuloma originating from the dura mater with secondary osseous invasion in an 11-year-old female child who presented with a swelling in the right parietal region. Magnetic resonance imaging demonstrated an enhancing mass with a wide dural attachment with a lytic lesion in the overlying skull...
October 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/29107666/jak2-mutated-langerhans-cell-histiocytosis-associated-with-primary-myelofibrosis-treated-with-ruxolitinib
#5
Arturo Bonometti, Filippo Bagnoli, Daniele Fanoni, Luigia Venegoni, Laura Corti, Paola Bianchi, Elena Maria Elli, Giuseppe Isimbaldi, Vincenzo L'Imperio, Gianluca Nazzaro, Emanuela Passoni, Emilio Berti
The pathogenesis and cellular origin of Langerhans cell histiocytosis (LCH) are debated. Recently, mutations on MAPK and PI3K pathways have been linked to disrupted cell proliferation in LCH. Janus Kinase 2 (JAK2) mutations play the same role in Philadelphia-negative chronic myeloproliferative neoplasms. We describe the case of a patient affected by JAK2-positive Primary Myelofibrosis (PMF) who developed a clonally related LCH while in treatment with Ruxolitinib. JAK-inhibitors are well known to affect function and differentiation of different hematological lineages, including mononuclear phagocytes precursors...
October 28, 2017: Human Pathology
https://www.readbyqxmd.com/read/29107340/langerhans-cell-histiocytosis-a-neoplastic-disorder-driven-by-ras-erk-pathway-mutations
#6
REVIEW
Gary Tran, Thy N Huynh, Amy S Paller
Langerhans cell histiocytosis (LCH) is a disorder of myeloid neoplasia of dendritic cells that affects 1 in 200,000 children <15 years of age and even fewer adults. LCH presents with a spectrum of clinical manifestations. High-risk stratification is reserved for infiltration of blood, spleen, liver, and lungs. After decades of debate on the disease pathogenesis, a neoplastic mechanism is now favored on the basis of LCH cell clonality, rare cases of familial clustering, and recent evidence of mutations involving the Ras/Raf/MEK (mitogen-activated protein kinase kinase)/ERK (extracellular signal-regulated kinase) pathway in lesional biopsy specimens...
October 26, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29099362/oral-manifestations-of-systemic-disease
#7
Heidi L Gaddey
On examination, the oral cavity may exhibit manifestations of underlying systemic disease and serve as an indicator of overall health. Systemic diseases with oral findings include autoimmune, hematologic, endocrine, and neoplastic processes. Autoimmune disease may manifest as oral ulcerations, changes in the salivary and parotid glands, and changes in the tongue. Patients with hematologic illnesses may present with gingival bleeding or tongue changes such as glossitis, depending on the etiology. Oral changes associated with endocrine illness are variable and depend on the underlying condition...
November 2017: General Dentistry
https://www.readbyqxmd.com/read/29096034/cns-erdheim-chester-disease-a-challenge-to-diagnose
#8
Zenggang Pan, Bette K Kleinschmidt-DeMasters
Erdheim-Chester disease (ECD) is a rare nonLangerhans cell histiocytosis. Although approximately 50% of cases eventually involve the central nervous system (CNS), the CNS has seldom been reported as the initial biopsy site. The diagnosis of CNS ECD can be challenging due to morphologic overlap with reactive histiocytic proliferation, Langerhans cell histiocytosis (LCH), and extranodal Rosai-Dorfman disease (RDD). We present 3 cases from our files that illustrate the protean manifestations of ECD. Case 1 was a 47-year-old man with ataxia, dysarthria, and intermittent ophthalmoplegia whose cerebellar biopsy had shown only profuse, nonspecific Rosenthal fiber-rich piloid gliosis; ECD was diagnosed only at autopsy...
December 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29083024/pulmonary-langerhans-cell-histiocytosis-in-adults
#9
Elżbieta Radzikowska
Pulmonary Langerhans' cell histiocytosis (PLCH) is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow derived Langerhans' cells, which carry mutations of BRAF gene and/or NRAS, KRAS and MAP2K1 genes. It occurs predominantly in young smokers, without gender predominance. The disease is characterised by formation of eosinophilic granulomas with the presence of Langerhans' cells infiltrating and destroying distal airways. High-resolution computed tomography of the chest (HRCT) plays an outstanding role in PLCH diagnosis...
October 30, 2017: Advances in Respiratory Medicine
https://www.readbyqxmd.com/read/29079636/alk-fusions-in-a-wide-variety-of-tumor-types-respond-to-anti-alk-targeted-therapy
#10
Jeffrey S Ross, Siraj M Ali, Omotayo Fasan, Jared Block, Sumanta Pal, Julia A Elvin, Alexa B Schrock, James Suh, Sahar Nozad, Sungeun Kim, Hwa Jeong Lee, Christine E Sheehan, David M Jones, Jo-Anne Vergilio, Shakti Ramkissoon, Eric Severson, Sugganth Daniel, David Fabrizio, Garrett Frampton, Vince A Miller, Philip J Stephens, Laurie M Gay
BACKGROUND: Genomic fusions of the anaplastic lymphoma kinase gene (ALK) are a well-established therapy target in non-small cell lung cancer (NSCLC). From a survey of 114,200 clinical cases, we determined the prevalence of ALK rearrangements (rALK) in non-NSCLC tumors and report their responsiveness to therapies targeting ALK. MATERIALS AND METHODS: Comprehensive genomic profiling of 114,200 relapsed and metastatic malignancies, including both solid tumors and hematolymphoid cancers, was performed using a hybrid-capture, adaptor ligation-based next-generation sequencing assay...
October 27, 2017: Oncologist
https://www.readbyqxmd.com/read/29058834/-difficult-diagnosis-of-erdheim-chester-disease-revealed-by-central-diabetes-insipidus
#11
W Alaya, B Zantour, W Ben Salem, W Chebbi, H-M Sfar
The Erdheim Chester disease is a rare form of non Langerhans cell histiocytosis. Its rarity and its unspecific clinical presentation, make that its diagnosis is often delayed. We report the case of a 50 years old female who has an Erdheim Chester disease, revealed by a central diabetes insipidus with thickening of the pituitary stalk, with associated gonadotropin deficiency. The Erdheim Chester disease was suspected because of the association with other evocative systemic lesions: eyelid xanthelasmas and bone lesions in metaphyseal-diaphyseal region of the upper and lower ends of both femurs and tibias on bone scan...
October 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/29052290/pulmonary-langerhans-cell-histiocytosis-de-novo-after-lung-transplantation
#12
Javier Gómez-Román, María Teresa Zarrabeitia, Ana Santurtún, José Antonio Parra, Ainara Azueta Etxebarría, Jose M Cifrian, Javier Freire Salinas
A pulmonary Langerhans cell histiocytosis is presented in a 40 year-old woman two years after bilateral lung transplantation for emphysema without any signs of Langerhans cells proliferation in the explanted lungs. A microsatellite molecular analysis showed the proliferating cells were generated in a recipient cellular clone. The patient did not quit smoking after transplantation. No signs of disease were detected in the implanted lungs before surgery. Strict control of immunosupressive drug levels stabilized the disease...
October 20, 2017: Pathology International
https://www.readbyqxmd.com/read/29051154/incidental-langerhans-cell-histiocytosis-of-the-colon-with-braf-p-v600e-mutation
#13
Jacqueline M Cortazar, Annette S Kim
No abstract text is available yet for this article.
October 19, 2017: Blood
https://www.readbyqxmd.com/read/29046979/medical-management-of-pulmonary-hypertension-with-unclear-and-or-multifactorial-mechanisms-group-5-is-there-a-role-for-pulmonary-arterial-hypertension-medications
#14
REVIEW
Jason Weatherald, Laurent Savale, Marc Humbert
PURPOSE OF REVIEW: The purpose of this review was to outline the mechanisms and to review recent literature on pulmonary arterial hypertension (PAH) medications in group 5 pulmonary hypertension (PH). RECENT FINDINGS: The first steps in management are to understand the mechanisms and hemodynamic profile and to exclude chronic thromboembolic disease. Recent studies in the past 5 years have found that PAH medications may improve hemodynamics in patients with pre-capillary pulmonary hypertension due to sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, and myeloproliferative disorders with dasatinib-induced PH...
October 18, 2017: Current Hypertension Reports
https://www.readbyqxmd.com/read/29044633/extensive-nail-changes-in-a-toddler-with-multisystemic-langerhans-cell-histiocytosis
#15
Vimal Kumar, Dhanalakshmi Angappan, Julius Scott, Deenadayalan Munirathnam, Mukul Vij, Naresh Shanmugam
Langerhans cell histiocytosis (LCH) is a multisystem disorder involving various organs. Nail changes in LCH are extremely rare. We present this case report of extensive nail changes in an 18-month-old child with multisystem LCH.
October 17, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/29043209/perianal-langerhans-cell-histiocytosis-a-rare-presentation-in-an-adult-male
#16
Asmaa Gaber Abdou, Doha MaherTaie
Langerhans cell histiocytosis (LCH) is a rare disease characterized by a proliferation of cells that show immunophenotypic and ultrastructural similarities with antigen-presenting Langerhans cells of mucosal sites and skin. LCH in adults is rare, and there are still many undiagnosed/misdiagnosed patients. We describe LCH involvement of the perianal region of a 33-year-old male with a previous history of diabetes insipidus. The differential diagnosis and all the reported cases of LCH of the perianal skin involvement since its description in 1984 till 2016 are discussed...
July 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/29026568/a-rare-case-of-cd1a-negative-langerhans-cell-histiocytosis-of-the-central-nervous-system-in-a-child
#17
Priscilla Powell, Gaile Vitug, Fernando Castro-Silva, Anish Ray
Langerhans cell histiocytosis is a dendritic cell disorder with a wide spectrum of severity and presentations. Histopathology typically demonstrates a proliferation of Langerhans cells and a lymphohistiocytic inflammatory infiltrate with eosinophils. The diagnosis is supported by immunohistochemistry with the cell markers S100, CD1a, CD68, and Langerin [Blood, 126, 2015, 26 and N Engl J Med, 331, 1994, 154].
October 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29021671/atlantoaxial-langerhans-cell-histiocytosis-radiographic-characteristics-and-corresponding-prognosis-analysis
#18
Lihua Zhang, Liang Jiang, Huishu Yuan, Zhongjun Liu, Xiaoguang Liu
BACKGROUND: Langerhans cell histiocytosis (LCH) may affect atlas and axis, and there were very few published cases describing a characteristic of LCH of atlantoaxial. OBJECTIVE: The objective of the study is to investigate the image manifestations of atlantoaxial LCH to improve the in-depth comprehension on it. MATERIALS AND METHODS: A retrospective study was done of computed tomography (CT) and magnetic resonance imaging in atlas and axis and prognosis was analyzed...
July 2017: Journal of Craniovertebral Junction and Spine
https://www.readbyqxmd.com/read/29017411/occult-langerhans-cell-histiocytosis-in-clear-cell-renal-cell-carcinoma
#19
Hee Jung Kwon, Phil Hyun Song, Mi Jin Gu
Langerhans cell histiocytosis is a rare disease that is characterized by a localized or systemic proliferation of Langerhans dendritic cells and a wide spectrum of clinical presentations. We experienced an unusual case of occult Langerhans cell histiocytosis associated with clear cell renal cell carcinoma. A 62-year-old man underwent a partial nephrectomy for left renal mass. Histologic examination showed nests of clear cells with surrounding thin-walled vessel that were suggestive of clear cell renal cell carcinoma...
October 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29017309/langerhans-cell-histiocytosis-of-the-thoracic-spine-in-an-adult
#20
Myeong Cheol Kim, Sun Hee Sung, Yongjae Cho
We report a case of a 45-year-old man with a complaint of both leg weakness and hypoesthesia. Radiological evaluation revealed an osteolytic lesion of the ninth thoracic vertebra. The patient underwent posterior corpectomy with total excision of the tumor, mesh cage insertion with posterior screw fixation and subsequent radiotherapy. Histology confirmed the diagnosis of Langerhans cell histiocytosis (LCH). This case report presents the diagnostic work-up, histopathological evaluation, and the treatment procedures of rare LCH in the thoracic spine...
September 2017: Korean Journal of Spine
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