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https://www.readbyqxmd.com/read/28731669/-langerhans-cell-histiocytosis-with-vertebral-involvement-and-soft-tissue-extension-clinical-case
#1
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
#2
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/28716520/modeling-color-preference-using-color-space-metrics
#3
Karen B Schloss, Laurent Lessard, Chris Racey, Anya C Hurlbert
Studying color preferences provides a means to discover how perceptual experiences map onto cognitive and affective judgments. A challenge is finding a parsimonious way to describe and predict patterns of color preferences, which are complex with rich individual differences. One approach has been to model color preferences using factors from metric color spaces to establish direct correspondences between dimensions of color and preference. Prior work established that substantial, but not all, variance in color preferences could be captured by weights on color space dimensions using multiple linear regression...
July 14, 2017: Vision Research
https://www.readbyqxmd.com/read/28700967/eosinophilic-granuloma-at-the-cerebellopontine-angle-in-an-adult-a-rare-case-report-and-literature-review
#4
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/28690898/delayed-treatment-response-in-a-neonate-with-multisystem-langerhans-cell-histiocytosis-case-report-and-review-of-literature
#5
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/28688556/pediatric-langerhans-cell-histiocytosis-of-the-lateral-skull-base
#6
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/28655945/a-case-of-refractory-langerhans-cell-histiocytosis-complicated-with-hemophagocytic-lymphohistiocytosis-rescued-by-cord-blood-transplantation-with-reduced-intensity-conditioning
#7
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/28638562/a-rare-case-of-oral-multisystem-langerhans-cell-histiocytosis
#8
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/28622183/cyclin-d1-is-expressed-in-neoplastic-cells-of-langerhans-cell-histiocytosis-but-not-reactive-langerhans-cell-proliferations
#9
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/28614815/a-case-of-pulmonary-langerhans-cell-sarcoma-simultaneously-diagnosed-with-cutaneous-langerhans-cell-histiocytosis-studied-by-whole-exome-sequencing
#10
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/28606227/-clinical-features-and-prognosis-of-langerhans-cell-histiocytosis-in-children-an-analysis-of-34-cases
#11
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/28601515/repeated-pulse-exposures-to-lambda-cyhalothrin-affect-the-behavior-physiology-and-survival-of-the-damselfly-larvae-ischnura-graellsii-insecta-odonata
#12
Simone Finotello, Alexander Feckler, Mirco Bundschuh, Frank Johansson
Damselflies form an essential part of the aquatic and terrestrial food web. Pesticides may, however, negatively affect their behavior, physiology, and survival. To assess this, a 42-day-lasting bioassay was conducted, during which damselfly larvae (Ischnura graellsii; n = 20) were repeatedly exposed to lambda-cyhalothrin (3 days at; 0, 10, 50, 250, 1250, and 6250ng LCH L(-1)), followed by recovery phases (4 days) in pesticide-free medium for six weeks. This exposure design was used to simulate frequent runoff events in the field...
June 8, 2017: Ecotoxicology and Environmental Safety
https://www.readbyqxmd.com/read/28596665/langerhans-cell-histiocytosis-lch-of-the-tonsil-in-adult-patient-an-uncommon-disease-at-an-uncommon-site
#13
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/28582647/inflammatory-serum-cytokines-and-chemokines-increase-associated-with-the-disease-extent-in-pediatric-langerhans-cell-histiocytosis
#14
Akira Morimoto, Yukiko Oh, Sachie Nakamura, Yoko Shioda, Tomomi Hayase, Toshihiko Imamura, Kazuko Kudo, Shinsaku Imashuku
OBJECTIVE: Langerhans cell histiocytosis (LCH) is characterized by immature dendritic cell proliferation, infiltration of LCH lesions by various inflammatory cells, and a lesional cytokine storm. It is classified into three groups on the basis of disease extent, namely, multisystem with risk-organ involvement (MS+), multisystem without risk-organ involvement (MS-), and single-system (SS) disease. We comprehensively analyzed whether serum levels of cytokines/chemokines reflect the disease extent...
September 2017: Cytokine
https://www.readbyqxmd.com/read/28581023/effects-of-a-daily-3-meal-pattern-with-different-dietary-protein-contents-on-pig-growth-performance-carcass-and-muscle-quality-traits
#15
Xin Wu, Chunyan Xie, Cimin Long, Jun Li, Xihong Zhou, Zhiyong Fan, Francois Blachier, Yulong Yin
BACKGROUND: Recent research related to phase-feeding programs for pig nutrition do not always account for the variation among individuals, and feeds are usually formulated to optimize the performance of the whole pig population. This study aimed at measuring the effects of a daily 3-meal pattern with different dietary protein contents on pig growth performance, carcass and muscle quality traits. RESULTS: The results showed that compared with the 3C treatment, ADG of pigs in the HCL treatment increased by 14...
June 5, 2017: Journal of the Science of Food and Agriculture
https://www.readbyqxmd.com/read/28566492/functional-evidence-for-derivation-of-systemic-histiocytic-neoplasms-from-hematopoietic-stem-progenitor-cells
#16
Benjamin H Durham, Damien Roos-Weil, Claude Baillou, Fleur Cohen-Aubart, Akihide Yoshimi, Makoto Miyara, Matthias Papo, Zofia Hélias-Rodzewicz, Nathalie Terrones, Neval Ozkaya, Ahmet Dogan, Raajit Rampal, Fanny Urbain, Lucie Le Fèvre, Eli L Diamond, Christopher Y Park, Thomas Papo, Frédéric Charlotte, Guy Gorochov, Valérie Taly, Olivier A Bernard, Zahir Amoura, Omar Abdel-Wahab, François M Lemoine, Julien Haroche, Jean-François Emile
Langerhans cell histiocytosis (LCH) and the non-LCH neoplasm Erdheim-Chester disease (ECD) are heterogeneous neoplastic disorders marked by infiltration of pathologic macrophage-, dendritic cell-, or monocyte-derived cells in tissues driven by recurrent mutations activating MAPK signaling. Although recent data indicate that at least a proportion of LCH and ECD patients have detectable activating kinase mutations in circulating hematopoietic cells and bone marrow-based hematopoietic progenitors, functional evidence of the cell of origin of histiocytosis from actual patient materials has long been elusive...
July 13, 2017: Blood
https://www.readbyqxmd.com/read/28562520/langerhans-cell-histiocytosis-mimicking-a-pott-puffy-tumor
#17
Luca Pasquini, Maria Camilla Rossi Espagnet, Giacomo Esposito, Rita De Vito, Raffaella Messina, Stefania Gaspari, Daniela Longo
Langherans' cell histiocytosis (LCH) is a rare disease mostly affecting children in the first decade of life. As clinical presentation is extremely heterogenous, a prompt diagnosis may be challenging, sometimes leading to a diagnostic delay, especially when the disease involves a single site. Herein, we report a case of a child with an unusual presentation of (LCH) mimicking a Pott puffy tumor with extracranial and epidural abscesses, surgically treated. Through this unique case we summarize possible manifestations of LCH with bone involvement and we underline the importance of considering possible complications due to bone erosions such as infection, to avoid a misdiagnosis...
May 29, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28551613/erdheim-chester-disease-comprehensive-review-of-molecular-profiling-and-therapeutic-advances
#18
REVIEW
Faysal Haroun, Kristen Millado, Imad Tabbara
The revised 2016 World Health Organization classification introduced Erdheim-Chester disease (ECD) as a provisional entity within the histiocytic and dendritic cell neoplasms separate from the juvenile xanthogranuloma family based on distinct molecular features. However, evolving knowledge regarding the molecular and genetic aberrations in addition to common clinical features of ECD support the classification of ECD together with Langerhans cell histiocytosis (LCH). Accordingly, ECD can be thought of as an inflammatory myeloid clonal disorder based on the detection of various activating mutations along the mitogen activated protein kinase-extracellular signal regulated kinase (MAPK-ERK) pathway with most notable variant being a valine to a glutamic acid substitution at amino acid 600 in the B-rapidly accelerated fibrosarcoma protein (BRAFV600E)...
June 2017: Anticancer Research
https://www.readbyqxmd.com/read/28550040/kras-g12d-expression-in-lung-resident-myeloid-cells-promotes-pulmonary-lch-like-neoplasm-sensitive-to-statin-treatment
#19
Tamihiro Kamata, Susan Giblett, Catrin Pritchard
Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm associated with somatic mutations in the genes involved in the RAF/MEK/ERK signaling pathway. Recently, oncogenic mutations in NRAS/KRAS, upstream regulators of the RAF/MEK/ERK pathway, have been reported in pulmonary, but not in non-pulmonary, LCH cases, suggesting organ-specific contribution of oncogenic RAS to LCH pathogenesis. Using a mouse model expressing KRAS(G12D) in the lung by nasal delivery of adenoviral Cre, here we show that KRAS(G12D) expression in lung-resident myeloid cells induces pulmonary LCH-like neoplasms comprised of pathogenic CD11c(high)F4/80+CD207+ cells...
May 26, 2017: Blood
https://www.readbyqxmd.com/read/28533655/-18-f-fdg-pet-ct-in-localizing-additional-cns-lesion-in-a-case-of-langerhans-cell-histiocytosis-determining-accurate-extent-of-the-disease
#20
Shamim Ahmed Shamim, Sarthak Tripathy, Anirban Mukherjee, Chandrasekhar Bal, Madhavi Tripathi
Central nervous system involvement is a rare manifestation of Langerhans cell histiocytosis (LCH), with bone and skin lesions being more frequent. MR remains the investigation of choice for localizing brain lesions. However, due to poor sensitivity of MRI in detecting osseous and pulmonary lesions, it is not used routinely in staging purposes until and unless indicated. We hereby discuss a case of 6-year-old boy of LCH who was referred for 18-F-FDG PET/CT for staging and knowing the extent of the disease, but a lesion in hypothalamus was picked up incidentally on FDG PET-CT study that was confirmed by MRI...
April 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
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