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Langerhans histiocytosis

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https://www.readbyqxmd.com/read/28731671/-langerhans-cell-histiocytosis-presenting-as-isolated-adenitis-in-an-infant-case-report
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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...
June 29, 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
#8
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
https://www.readbyqxmd.com/read/28690898/delayed-treatment-response-in-a-neonate-with-multisystem-langerhans-cell-histiocytosis-case-report-and-review-of-literature
#9
Amitabh Singh, Anirban Mandal, Lavleen Singh, Sataroopa Mishra, Ankita Patel
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, which are part of the mononuclear phagocytic system. The disorder varies in terms of the extent of the disease, its natural course and patient outcomes. While skin rashes are a common presentation of neonatal LCH, other systems or organs may also be involved. Delays in the diagnosis of neonatal LCH may occur due to its non-specific presentation and a lack of awareness of the condition among doctors. We report a two-month-old male neonate who presented to the Chacha Nehru Bal Chikitsalya hospital, New Delhi, India, in 2016 after the onset of pulmonary symptoms...
May 2017: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/28690524/erdheim-chester-disease-case-report-with-aggressive-multisystem-manifestations-and-review-of-the-literature
#10
Sultan Alotaibi, Osama Alhafi, Hatem Nasr, Khalid Eltayeb, Ghaleb Elyamany
Erdheim-Chester disease (ECD) is an extremely rare and aggressive form of non-Langerhans cell histiocytosis. ECD usually presents with bone pain in adults aged 40-60. Its etiology is unknown but it is thought to be either a reactive or neoplastic disorder. Recently, mutation of the proto-oncogene BRAF (BRAFV600E) has been found in more than 50% of cases. The multisystemic form of ECD is associated with significant morbidity, which may arise due to histiocytic infiltration of critical organ systems. The common sites of involvement are the skeleton, central nervous system, cardiovascular system, lungs, retroperitoneum, and skin...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28689173/current-understanding-and-management-of-pulmonary-langerhans-cell-histiocytosis
#11
Robert Vassallo, Sergio Harari, Abdellatif Tazi
Pulmonary Langerhans cell histiocytosis (PLCH) is a diffuse lung disease that usually affects young adult smokers. PLCH affects different lung compartments; bronchiolar, interstitial and pulmonary vascular dysfunction may coexist to varying extents, resulting in diverse phenotypes. Analyses of PLCH tissues have identified activating mutations of specific mitogen-activated protein kinases (BRAF(V600E) and others). The current consensus is that PLCH represents a myeloid neoplasm with inflammatory properties: the myeloid tumour cells exhibit surface CD1a expression and up to 50% of the cells harbour activating BRAF or other MAPK mutations...
July 8, 2017: Thorax
https://www.readbyqxmd.com/read/28688556/pediatric-langerhans-cell-histiocytosis-of-the-lateral-skull-base
#12
Ananya Majumder, Cameron C Wick, Rebecca Collins, Timothy N Booth, Brandon Isaacson, J Walter Kutz
OBJECTIVE: Describe the presentation, imaging characteristics, management, and outcomes of pediatric patients with Langerhans cell histiocytosis (LCH) of the temporal bone. METHODS: A retrospective chart review was performed between 2000 and 2014 at a single tertiary care children's hospital. Fourteen patients were identified with a diagnosis of LCH and involvement of the temporal bone. RESULTS: Ten patients were female and ten were Caucasian...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28680751/tocilizumab-in-patients-with-multisystem-erdheim-chester-disease
#13
Alvise Berti, Giulio Cavalli, Barbara Guglielmi, Riccardo Biavasco, Corrado Campochiaro, Alessandro Tomelleri, Roberto Nicoletti, Andrea Panzacchi, Marina Ferrarini, Lorenzo Dagna
Treatment of Erdheim-Chester disease (ECD), a rare non-Langerhans histiocytosis, relies on interferon-α, chemotherapeutic agents such as purine analogs, cytokine-blocking agents and BRAF inhibitors. Since interleukin (IL)-6 levels are elevated in serum and lesions of ECD patients, we evaluated the therapeutic efficacy and safety of IL-6 blockade with tocilizumab. We conducted an open-label, single-arm, phase II, prospective study of tocilizumab in three patients with multisystem ECD and poor tolerance/contraindications to IFN-α...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28679734/high-prevalence-of-myeloid-neoplasms-in-adults-with-non-langerhans-cell-histiocytosis
#14
Matthias Papo, Eli L Diamond, Fleur Cohen-Aubart, Jean-François Emile, Damien Roos-Weil, Nishant Gupta, Benjamin H Durham, Neval Ozkaya, Ahmet Dogan, Gary A Ulaner, Raajit Rampal, Jean-Emmanuel Kahn, Thomas Sené, Frédéric Charlotte, Baptiste Hervier, Caroline Besnard, Olivier A Bernard, Catherine Settegrana, Nathalie Droin, Zofia Hélias-Rodzewicz, Zahir Amoura, Omar Abdel-Wahab, Julien Haroche
Erdheim-Chester Disease (ECD) is a rare non-Langerhans Cell histiocytosis that most commonly affects adults and is driven by a high frequency of mutations in BRAF, MAP2K1, and kinases promoting MAP kinase signaling. Due to the relative rarity of ECD, key clinical features of the disease may not be well defined. Across a multi-institutional cohort of 189 patients with ECD and ECD overlapping with Langerhans Cell Histiocytosis (so-called Mixed Histiocytosis (MH)), we identified an unexpected and heretofore undescribed frequent occurrence of myeloid neoplasms amongst patients with ECD and MH...
July 5, 2017: Blood
https://www.readbyqxmd.com/read/28670259/langerhans-cell-histiocytosis-followed-by-folliculotropic-mycosis-fungoides
#15
Izabela Błażewicz, Małgorzata Sokołowska-Wojdyło, Agnieszka Piekarska, Alicja Sadowska-Klasa, Anna Kowalczyk, Monika Konczalska, Berenika Olszewska, Wioletta Barańska-Rybak, Wojciech Biernat, Roman J Nowicki
No abstract text is available yet for this article.
June 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28664935/mutually-exclusive-recurrent-kras-and-map2k1-mutations-in-rosai-dorfman-disease
#16
Sofia Garces, L Jeffrey Medeiros, Keyur P Patel, Shaoying Li, Sergio Pina-Oviedo, Jingyi Li, Juan C Garces, Joseph D Khoury, C Cameron Yin
Rosai-Dorfman disease is a histiocytic disorder with a poorly defined pathogenesis. Recent molecular studies have revealed recurrent mutations involving genes in the MAPK/ERK pathway in Langerhans cell histiocytosis and Erdheim-Chester disease. However, cases of Rosai-Dorfman disease have rarely been assessed. We performed next-generation sequencing to assess 134 genes on 21 cases of Rosai-Dorfman disease, including 13 women and 8 men with a median age of 43 years (range, 3-82). In all, 13 had extranodal, 5 had nodal, and 3 had coexistent nodal and extranodal disease...
June 30, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28664013/erdheim-chester-disease-involving-the-central-nervous-system-with-the-unique-appearance-of-a-coated-vertebral-artery
#17
Hime Suzuki, Masahiko Wanibuchi, Katsuya Komatsu, Yukinori Akiyama, Takeshi Mikami, Shintaro Sugita, Tadashi Hasegawa, Mitsunori Kaya, Kohichi Takada, Nobuhiro Mikuni
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. It is characterized by multiple xanthogranulomatous masses throughout the body, predominantly in the tibia. One of the characteristic radiological findings of the lesions associated with ECD is a "coated artery," which is often observed in the aorta. Although approximately one-fourth of ECD cases involve the central nervous system (CNS), an intracranial-coated artery has only been reported in four cases. We report a case of ECD that involves the CNS and has the unique appearance of a coated vertebral artery (VA)...
October 2016: NMC Case Rep J
https://www.readbyqxmd.com/read/28655945/a-case-of-refractory-langerhans-cell-histiocytosis-complicated-with-hemophagocytic-lymphohistiocytosis-rescued-by-cord-blood-transplantation-with-reduced-intensity-conditioning
#18
Kana Washio, Michiko Muraoka, Kiichiro Kanamitsu, Megumi Oda, Akira Shimada
 We diagnosed a female infant with Langerhans cell histiocytosis (LCH) who was refractory to conventional chemotherapy. She showed refractory inflammation that was complicated with hemophagocytic lymphohistiocytosis (HLH) during LCH chemotherapy; therefore, we changed the protocol to HLH2004 (dexamethasone, cyclosporine A and VP16). However, there were no signs of hematological recovery. We therefore performed cord blood transplantation with reduced-intensity conditioning, and she achieved complete remission for over 2 years...
June 2017: Acta Medica Okayama
https://www.readbyqxmd.com/read/28649310/lung-ultrasound-has-limited-diagnostic-value-in-rare-cystic-lung-diseases-a-cross-sectional-study
#19
Jesper Rømhild Davidsen, Elisabeth Bendstrup, Daniel P Henriksen, Ole Graumann, Christian B Laursen
Background: Lung ultrasound (LUS) used to identify interstitial syndrome (IS) and pleural thickening related to diffuse parenchymal lung disease (DPLD) has shown significant correlations with ground glass opacity (GGO) on high-resolution computed tomography (HRCT). However, the applicability of LUS in patients with DPLD subtypes as rare cystic lung diseases has not previously been investigated. This study aimed to observe if distinctive LUS findings could be found in patients with lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), and Birt-Hogg-Dubé syndrome (BHDS)...
2017: European Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28640106/the-outcome-of-eosinophilic-granuloma-involving-unilateral-atlantoaxial-joint-a-case-report-and-literature-review
#20
REVIEW
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)
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