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Histiocytosis

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https://www.readbyqxmd.com/read/28645353/actualit%C3%A3-s-autour-de-la-prise-en-charge-des-diab%C3%A3-tes-insipides-centraux-management-of-central-diabetes-insipidus-in-2016
#1
H Lasolle, F Borson-Chazot
Diabetes insipidus is a syndrome that associates both hypotonic polyuria and polydipsia, due to insufficient or ineffective arginine vasopressin (AVP) synthesis, or to AVP resistance. The diagnosis between central/renal origin, or an abnormal thirst regulation (primary polydipsia) is required to organize an adapted management. Because water deprivation tests are not reliable, it's often based on medical history, response to treatment and MRI. Copeptin is an AVP precursor which could be very helpful for the diagnosis...
October 2016: Annales D'endocrinologie
https://www.readbyqxmd.com/read/28640106/the-outcome-of-eosinophilic-granuloma-involving-unilateral-atlantoaxial-joint-a-case-report-and-literature-review
#2
Yu Song, Wen Geng, Tao Guo, Yong Gao, Yukun Zhang, Shuai Li, Kun Wang, Ji Tu, Cao Yang
RATIONALE: Solitary eosinophilic granuloma (EG), the most benign, common form of Langerhans cell histiocytosis, has a self-limiting process and is associated with a good prognosis. Immobilization is recommended as the first treatment strategy for solitary EG, although the treatment protocols are still controversial. Radiotherapy and surgery are secondary treatment choices. Lesions of the upper cervical spine react differently to treatment because of their specific anatomical and motor features...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28638562/a-rare-case-of-oral-multisystem-langerhans-cell-histiocytosis
#3
Maria-Teresa Facciolo, Francesco Riva, Patrizia Gallenzi, Romeo Patini, Domenico Gaglioti
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue and often affects children and young men. A 29 years-old Caucasian man was referred to the Oral Surgery Unit of George Eastman Hospital - Umberto I teaching hospital, with third degree mobility of teeth belonging to second, third and fourth quadrant. Panoramic radiograph showed multiple radiolucent areas with well demarcated borders on the right and left site of the mandible and on the left site of the maxilla...
June 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28633156/generalized-indeterminate-cell-histiocytosis-presenting-as-eroded-papules-and-crusts
#4
Yaping Li, Harrison Xiao Bai, Chang Su, Guiying Zhang
Indeterminate cell histiocytosis (ICH) is a very rare histiocytic disease. We reported a case of generalized ICH presenting as eroded papules and crusts, accompanied with intermittent fever, pulmonary disorder, lymphadenectasis in the mediastinum, and the retroperitoneum. The histopathology indicated a neoplasm that was composed of small nests of large cells with ample pale cytoplasm. Immunohistochemistry results and the absence of Birbeck granules led us to the diagnosis of ICH. Four months later, the patient died of anemia, ascites, and swelling of the limbs...
July 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28630846/chronic-recurrent-multifocal-osteomyelitis-a-case-report-with-atypical-presentation
#5
Miguel Pádua Figueiredo, Marco Pato, Fernando Amaral
INTRODUCTION: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory condition. The clinical picture consists of sterile osteomyelitis, typically with multiple-site lesions in the metaphysis of long bones and not uncommonly, symmetrical bone involvement. It is a poorly understood entity, whose prognosis, etiology and ideal treatment are still controversial. The authors report a case of unifocal presentation with an atypical location. CASE REPORT: A previously healthy 12-year-old Caucasian girl came to our institution due to progressive pain on her left thigh for the previous 3 months...
January 2017: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28626593/langerhans-cell-sarcoma-a-case-report-demonstrating-morphological-and-immunophenotypical-variability-within-a-single-lesion
#6
Rasika Singh, Charles Edward Keen, Christopher Stone, Patrick Sarsfield
Langerhans cells are antigen presenting dendritic cells and tumours arising from these are rare. The tumours arising from these dendritic cells are divided into two categories according to a WHO classification: Langerhans cell histiocytosis and Langerhans cell sarcoma. It is the degree of atypia and clinical aggressiveness that distinguishes the two subtypes. Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells which can occur in skin, bone marrow, lymph nodes, spleen, liver, and lung...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28623120/association-between-ischemic-stroke-and-erdheim-chester-disease-a-case-report-and-review-of-literature
#7
Guillaume Fargeot, Sabrina Stefanizzi, Sebastien Depuydt, Frederic Klapczynski, Alain Ameri
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by a xanthogranulomatous infiltration of tissues by spumous histiocytes. Neurological involvement is frequent, but ischemic strokes have been exceptionally described. We report the case of a 68-year-old woman who presented with an acute ischemic stroke associated with a multisystemic disorder including insipidus diabetes, infiltration of the aorta and the carotid arteries, perirenal infiltration, aortitis, and lytic bone lesions...
June 13, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28622183/cyclin-d1-is-expressed-in-neoplastic-cells-of-langerhans-cell-histiocytosis-but-not-reactive-langerhans-cell-proliferations
#8
Vignesh Shanmugam, Jeffrey W Craig, Jason L Hornick, Elizabeth A Morgan, Geraldine S Pinkus, Olga Pozdnyakova
Langerhans cell histiocytosis (LCH) is characterized by frequent activating mutations involving the mitogen-activated protein kinase (MAPK) pathway. Therefore, downstream markers of MAPK pathway activation such as cyclin D1 may be useful as novel diagnostic markers of neoplasia in LCH. The goal of this study was to investigate cyclin D1 expression in LCH and reactive Langerhans cell accumulations using immunohistochemistry on archival tissue. All LCH cases tested (39/39) showed cyclin D1 expression in CD1a/Langerin cells...
June 15, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28617889/erdheim-chester-disease-presenting-with-histiocytic-colitis-and-cytokine-storm
#9
George P Christophi, Yeshika Sharma, Quader Farhan, Umang Jain, Ted Walker, Gregory S Sayuc, Deborah C Rubin
BACKGROUND: Non-Langerhans histiocytosis is a group of inflammatory lymphoproliferative disorders originating from non-clonal expansion of hematopoietic stem cells into cytokine-secreting dendritic cells or macrophages. Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans cell histiocytosis characterized by tissue inflammation and injury caused by macrophage infiltration and histologic findings of foamy histiocytes. Often ECD involves the skeleton, retroperitoneum and the orbits...
June 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28616240/metastatic-pancreatic-carcinoma-masquerading-as-cystic-lung-disease-a-rare-presentation
#10
Emily Stern, Taha Huseini, YiJin Kuok, Fiona Lake
This 52-year-old male ex-smoker presented with a six-month history of progressive breathlessness and weight loss. He deteriorated acutely, and was admitted with severe type 1 respiratory failure. Apart from diffuse coarse crackles on chest auscultation, physical examination was unremarkable. High-resolution computed tomography (HRCT) showed diffuse cystic changes throughout the lungs. A diagnosis of pulmonary Langerhans cell histiocytosis (PLCH) was considered. Further workup identified a coincidental pancreatic lesion of uncertain significance, which remained indeterminate on magnetic resonance imaging (MRI) and on positron emission tomography (PET)...
September 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28614815/a-case-of-pulmonary-langerhans-cell-sarcoma-simultaneously-diagnosed-with-cutaneous-langerhans-cell-histiocytosis-studied-by-whole-exome-sequencing
#11
Si-Wook Kim, Moon Ki Choi, Hye Sook Han, Hyojin Song, Youngil Koh, Seung-Myoung Son, Ok-Jun Lee, Ji Yeoun Lee, Ki Man Lee, Ki Hyeong Lee, Jihyun Kwon
Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS) are clonal proliferations of Langerhans-type cells. Unlike in LCH, the pathophysiology and clinical course of LCS are unclear due to its rarity. Here, we report the case of a 73-year-old male patient who was diagnosed with cutaneous LCH and pulmonary LCS at the same time. Pathological review of these 2 tumors revealed similar immunohistochemical findings. However, the tumor cells in LCS had more aggressive cytological features than those in LCH...
June 15, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28614806/review-of-rosai-dorfman-disease-new-insights-into-the-pathogenesis-of-this-rare-disorder
#12
Yanan Cai, Zhangzhen Shi, Yuansong Bai
Rosai-Dorfman disease (RDD) is a rare histiocytosis typically with bilateral painless cervical lymphadenopathy. Laboratory data are nonspecific, and the presence of emperipolesis in large foamy S-100+ CD1a- histiocytes is the prominent histologic feature. The pathogenesis of RDD still remains elusive. According to published studies, we propose that RDD cells might represent intermediate recruiting monocytes with differentiation blockade. Both disturbance of homoeostasis and inherent genomic alterations could contribute to initiation of the disorder through signal transduction...
June 15, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28614210/congenital-syphilis-of-bone-a-potential-mimicker-of-childhood-histiocytoses
#13
Youran Zou, Matthew A Marcus, C Guy Castles, Scott E Kilpatrick
Involvement of the skeletal system by congenital syphilis is well documented in the literature, chiefly in the form of radiologic studies, including periostitis, osteitis, and osteochondritis. Because congenital syphilis is generally recognized clinically, tissue biopsy is virtually never performed. Therefore, the histopathologic findings are less well documented and mostly exist in the older literature. We report herein the clinicoradiologic and pathologic features of a 2-month-old infant who initially presented with absence of left arm movement...
June 13, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28612007/erdheim-chester-disease-presenting-with-secondary-hypertension-as-a-result-of-bilateral-proximal-renal-artery-stenosis-a-case-report
#14
Farid Arman, Hania Shakeri, Niloofar Nobakht, Anjay Rastogi, Mohammad Kamgar
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis presenting most commonly with bone and central nervous system symptoms, including but not limited to bone pain and diabetes insipidus. We present a known case of ECD, which was referred for secondary hypertension workup and diagnosed with severe, proximal, bilateral renal artery stenosis.
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28610659/indeterminate-cell-histiocytosis-in-children-a-case-report
#15
A Sahuquillo-Torralba, M A Navarro-Mira, M Llavador-Ros, R Botella-Estrada
No abstract text is available yet for this article.
June 10, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28607780/homonymous-superior-quadrantanopia-due-to-erdheim-chester-disease-with-asymptomatic-pituitary-involvement
#16
Roaa Ridha Amer, Sara Mohammed Qubaiban, Eman Abdulkarim Bakhsh
Polyostotic sclerosing histiocytosis, also known as Erdheim-Chester disease (ECD), is a rare form of non-Langerhans histiocytosis. ECD has wide clinical spectrums which mainly affect skeletal, neurological, dermatological, retroperitoneal, cardiac, and pulmonary manifestations. Here we describe a case of ECD in a 45-year-old female who presented initially with bilateral knee pain and homonymous superior quadrantanopia progressed to ophthalmoplegia and complete visual loss of the left eye over a period of one year...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28606227/-clinical-features-and-prognosis-of-langerhans-cell-histiocytosis-in-children-an-analysis-of-34-cases
#17
Dan Li, Hui Li, Hong Shi
OBJECTIVE: To investigate the clinical features and prognosis of children with Langerhans cell histiocytosis (LCH). METHODS: A retrospective analysis was performed for the clinical data of 34 children with newly diagnosed LCH. RESULTS: The 34 children had a median age of 14.5 months (range: 22 d to 60 months). Of all 34 children, 23 were aged 0-2 years and 11 were aged >2 years. There were 17 children in the high-risk group and 17 in the low-risk group...
June 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28604476/pet-mr-in-the-assessment-of-pediatric-histiocytoses-a-comparison-to-pet-ct
#18
Andrew C Sher, Robert Orth, Kenneth McClain, Carl Allen, Shireen Hayatghaibi, Victor Seghers
PURPOSE: The aim of this study was to analyze the feasibility and diagnostic performance of F-FDG PET/MR compared with F-FDG PET/CT in a cohort of pediatric histiocytosis patients with regard to image quality, lesion detection, and FDG quantification. METHODS: Children with a diagnosis of Langerhans cell histiocytosis or Rosai-Dorfman disease were prospectively recruited. Seventeen PET/CT and PET/MR examinations were performed on 9 patients (mean age, 6.2 years) following a single-injection dual-imaging protocol...
June 9, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28596665/langerhans-cell-histiocytosis-lch-of-the-tonsil-in-adult-patient-an-uncommon-disease-at-an-uncommon-site
#19
Rachna Khera, Faiq Ahmed, Sudha Murthy, Krishna Mohan Mallavarapu
Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal neoplastic proliferation of normal antigen presenting cell (APC), the Langerhans cell. Most cases occur in childhood and the disease is rare in adults. LCH can involve solitary organ or can present as a multi-system disease in children. In adults, isolated pulmonary LCH is the commonest presentation. Tonsillar infiltration as a sole manifestation is extremely rare. We herewith report a case with isolated tonsillar involvement by LCH in an adult patient...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28592787/rare-gastric-lesions-associated-with-helicobacter-pylori-infection-a-histopathological-review
#20
Mee Joo
Helicobacter pylori infection is associated with chronic gastritis, peptic ulcer disease, gastric adenocarcinoma, and mucosa-associated lymphoid tissue lymphoma. However, some rare gastric lesions exhibiting distinctive histological features may also be associated with H. pylori infection, including lymphocytic gastritis, granulomatous gastritis, Russell body gastritis, or crystal-storing histiocytosis. Although diverse factors can contribute to their development, there is convincing evidence that H. pylori infection may play a pathogenic role...
June 5, 2017: Journal of Pathology and Translational Medicine
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