92. Vulvar and Oral Langerhans Cell Histiocytosis: A Pediatric Case Report
Langerhans cell histiocytosis (LCH) is an uncommon hematologic condition resulting from neoplastic proliferation of antigen-presenting cells with characteristics of Langerhans cells. These cells can accumulate in many organs including skin, bone, liver, brain and lung. Bone lesions are reported in 80% of cases of LCH. A diagnosis is made via histopathological analysis of lesions. LCH may present with single-system or multi-system lesions and lesions may be unifocal or multifocal. It is more common in children than adults. (Source: Journal of Pediatric and Adolescent Gynecology)
Source: Journal of Pediatric and Adolescent Gynecology - March 29, 2020 Category: OBGYN Authors: Jennifer Nestor, Donna Perruzza, Rafat Ahmed, Saifuddin Mama Source Type: research

Facial nerve paralysis as initial symptom of langerhans cell histiocytosis
Publication date: March–April 2020Source: Brazilian Journal of Otorhinolaryngology, Volume 86, Issue 2Author(s): Onur Ismı, Rabia Bozdogan Arpaci, Anıl Ozgur, Elvan Caglar Citak, Neslihan Eti, Tugce Puturgeli, Yusuf Vayisoglu (Source: Brazilian Journal of Otorhinolaryngology)
Source: Brazilian Journal of Otorhinolaryngology - March 27, 2020 Category: ENT & OMF Source Type: research

Langerhans Cell Histiocytosis With Vertebral Involvement Diagnosed and Treated Over the Last 15 Years in a Single Canadian Pediatric Academic Institution
We report 11 children with vertebral lesion of Langerhans cell histiocytosis (LCH) diagnosed and treated between 2000 and 2015. Vertebral lesions were usually present at LCH diagnosis. No child developed neurologic symptoms. Among 29 vertebral lesions, only 2 were unstable. Chemotherapy was used in all children but 3. A LCH recurrence was observed in 6 patients, involving vertebrae in 4 cases. All children were disease-free at their last follow-up. Sequelae were more often radiologic than clinical. Since potential recurrences and incomplete bone regeneration exist, discussion about optimal treatment and long-term follow-up...
Source: Journal of Pediatric Hematology Oncology - March 25, 2020 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Langerhans cell histiocytosis of an intra-mammary lymph node in an 18-year-old woman.
We describe an 18-year-old woman with several month's history of a 12 x 7 mm palpable mammary nodule, that was hypoechoic, with regular margins and vascularization areas by ultrasound. A fibroadenoma was hypothesized (American College of Radiology BI-RADS 3). A 14 G needle biopsy was performed, showing a LC proliferation suspected for LCH of a lymph node, with florid dermatopathic lymphadenopathy in differential diagnosis. The multidisciplinary team of the breast clinic decided to perform a lumpectomy and a diagnosis of LCH involving an intra-mammary lymph node was made. Langerhans cells (LC) are dendritic cells characteri...
Source: Pathologica - March 24, 2020 Category: Pathology Tags: Pathologica Source Type: research

Therapy Response in a Pediatric Patient With Extracutaneous Juvenile Xanthogranuloma Monitored by FDG PET/CT
We report here findings of a series of FDG PET/CT scans during the course of clofarabine therapy in a 12-year-old girl with extracutaneous juvenile xanthogranuloma. (Source: Clinical Nuclear Medicine)
Source: Clinical Nuclear Medicine - March 12, 2020 Category: Nuclear Medicine Tags: Interesting Images Source Type: research

Erdheim-Chester disease among neuroinflammatory syndromes: the case for precision medicine
Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis characterized by xanthogranulomatous infiltration typically affecting long bones, cardiovascular system, retroperitoneum, and lung, and that involves the CNS in 25%–50% of patients (table).1,2 Historically, establishing the diagnosis has been challenging, particularly in the absence of systemic abnormalities. Recent genomic studies have uncovered that approximately 50% of ECD tissue samples harbor a mutation in the BRAF gene,3 termed BRAFV600E, and that pointed to a neoplastic, rather than inflammatory, nature of the disease.4,5 (Source: Neurology ...
Source: Neurology Neuroimmunology and Neuroinflammation - March 2, 2020 Category: Neurology Authors: de la Fuente, M. I., Rosenblum, M. K., Diamond, E. L., Tabar, V. S., Omuro, A. Tags: All Clinical Neurology, All Medical/Systemic disease, All Oncology, Primary brain tumor, Chemotherapy-tumor Clinical/Scientific Notes Source Type: research

Genetic landscape of adult Langerhans cell histiocytosis with lung involvement
The clinical significance of the BRAFV600E mutation in adult Langerhans cell histiocytosis (LCH), including pulmonary Langerhans cell histiocytosis (PLCH), is not well understood. Similarly, the spectrum of molecular alterations involved in adult LCH has not been fully delineated. To address these issues, we genotyped a large number of adult LCH biopsies and searched for an association of identified molecular alterations with clinical presentation and disease outcome. Biopsies from 117 adult LCH patients, 83 with PLCH (median age 36.4 years, 56 females, 38 multisystem disease, 79 single system disease, 65 current smok...
Source: European Respiratory Journal - February 27, 2020 Category: Respiratory Medicine Authors: Jouenne, F., Chevret, S., Bugnet, E., Clappier, E., Lorillon, G., Meignin, V., Sadoux, A., Cohen, S., Haziot, A., How-Kit, A., Kannengiesser, C., Lebbe, C., Gossot, D., Mourah, S., Tazi, A. Tags: Original Articles: Rare lung disease Source Type: research

A new presentation of isolated cutaneous Rosai-Dorfman disease: Eruptive xanthoma-like lesions
Mohammed Fawzy El-Kamel, Mohamed Khaled Selim, Mamdouh Morsy Abdel GawadIndian Journal of Dermatology, Venereology, and Leprology 2020 86(2):158-161 Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a benign lympho-histiocytic proliferative disorder initially described with bilateral painless lymphadenopathy (90 %), fever, leukocytosis, elevated ESR, anemia, and polyclonal hypergammaglobulinemia (90 %). Extranodal forms occur in 43% of cases, the skin being the most common site. Around 10% of patients have skin lesions and in 3%, the disease is limited exclusively to the skin...
Source: Indian Journal of Dermatology, Venereology and Leprology - February 20, 2020 Category: Dermatology Authors: Mohammed Fawzy El-Kamel Mohamed Khaled Selim Mamdouh Morsy Abdel Gawad Source Type: research

Clinical Characteristics of Pediatric Patients With Sellar and Suprasellar Lesions Who Initially Present With Central Diabetes Insipidus: A Retrospective Study of 55 Cases From a Large Pituitary Center in China
Conclusion: For pediatric SSR lesions that first manifest as CDI, we should comprehensively consider clinical characteristics and imaging features to aid in their early differential diagnosis. Tumor markers and surgical histopathology are also great complements for the differential diagnosis. Additionally, various treatment strategies should be adopted according to different causes to improve the child's prognosis and quality of life. (Source: Frontiers in Endocrinology)
Source: Frontiers in Endocrinology - February 20, 2020 Category: Endocrinology Source Type: research

Langerhans cell histiocytosis: A case report with oral manifestations and the role of pediatric dentists in the diagnosis
Langerhans cell histiocytosis (LCH) is a benign disease that behaves malignantly. Early recognition and treatment of oral manifestation of LCH by pediatric dentist and other medical specialties is important to prevent further organ damage. AbstractLangerhans cell histiocytosis (LCH) is a benign disease that behaves malignantly. Early recognition and treatment of oral manifestation of LCH by pediatric dentist and other medical specialties is important to prevent further organ damage. (Source: Clinical Case Reports)
Source: Clinical Case Reports - February 15, 2020 Category: General Medicine Authors: Eman Hussein Hammouri, Hala Antoun Sweidan, Omar AShokaibi, Leen Al Omari Tags: CASE REPORT Source Type: research

[From fibrogenesis towards fibrosis: Pathophysiological mechanisms and clinical presentations].
Authors: Maria ATJ, Bourgier C, Martinaud C, Borie R, Rozier P, Rivière S, Crestani B, Guilpain P Abstract Fibrogenesis is a universal and ubiquitous process associated with tissue healing. The impairment of tissue homeostasis resulting from the deregulation of numerous cellular actors, under the effect of specific cytokine and pro-oxidative environments can lead to extensive tissue fibrosis, organ dysfunction and significant morbidity and mortality. This situation is frequent in internal medicine, since fibrosis is associated with most organ insufficiencies (i.e. cardiac, renal, or hepatic chronic failures)...
Source: Revue de Medecine Interne - February 14, 2020 Category: Internal Medicine Tags: Rev Med Interne Source Type: research

Erdheim-Chester disease: An in vivo human model of M ϕ activation at the crossroad between chronic inflammation and cancer.
Erdheim-Chester disease: An in vivo human model of Mϕ activation at the crossroad between chronic inflammation and cancer. J Leukoc Biol. 2020 Feb 14;: Authors: Cavalli G, Dagna L, Biavasco R, Villa A, Doglioni C, Ferrero E, Ferrarini M Abstract Erdheim-Chester disease (ECD) is a rare histiocytosis characterized by infiltration of multiple tissues by CD68+ foamy Mϕs (or 'histiocytes'). Clinical manifestations arise from mass-forming lesions or from tissue and systemic inflammation. ECD histiocytes harbor oncogenic mutations along the MAPK-kinase signaling pathway (BRAFV600E in more than half of the ...
Source: Journal of Leukocyte Biology - February 14, 2020 Category: Hematology Authors: Cavalli G, Dagna L, Biavasco R, Villa A, Doglioni C, Ferrero E, Ferrarini M Tags: J Leukoc Biol Source Type: research

Impact of graft-versus-host disease on the clinical outcome of allogeneic hematopoietic stem cell transplantation for non-malignant diseases.
In conclusion, acute and chronic GVHD, even if mild, was associated with reduced OS in patients receiving HSCT for NMD and effective strategies should, therefore, be implemented to minimize GVHD. PMID: 32052319 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 12, 2020 Category: Hematology Authors: Umeda K, Imai K, Yanagimachi M, Yabe H, Kobayashi M, Takahashi Y, Kajiwara M, Yoshida N, Cho Y, Inoue M, Hashii Y, Atsuta Y, Morio T, Inherited Disease Working Group of the Japan Society for Hematopoietic Cell Transplantation Tags: Int J Hematol Source Type: research

Intrahepatocellular crystal storing mimicking a clinical liver disease during monoclonal gammopathy: report of a case and review of the literature.
We present an unusual case of liver involvement in monoclonal gammopathy with generalized crystal-storing histiocytosis (G-CSH).A bone marrow storage disease was diagnosed in a 79-year-old man with monoclonal gammopathy of uncertain significance (MGUS). The patient presented with pleural effusion, an osteolytic lesion of the humerus, and an increase of aspartate transaminase and cholestatic markers that raised the clinical suspect of liver disease. A second bone marrow biopsy confirmed the diagnosis of MGUS with a histiocytic component suggestive for G-CSH.Liver biopsy showed an unremarkable histology, no significant infla...
Source: Ultrastructural Pathology - February 10, 2020 Category: Pathology Authors: Riefolo M, Malvi D, Bertuzzi C, Sabattini E, Valente S, Pasquinelli G, D'Errico A, Vasuri F Tags: Ultrastruct Pathol Source Type: research

Rosai Dorfman disease of the spine causing lumbosacral radiculopathy: A case report
Publication date: Available online 24 January 2020Source: Journal of Clinical Orthopaedics and TraumaAuthor(s): Prasad Patgaonkar, Alpeshkumar Thumbadia, Nandan Marathe, Vivek PatelAbstractRosai Dorfman disease (RDD) also known as sinus histiocytosis with massive lymphadenopathy (SHML) is a rare non-malignant proliferative disorder of unknown etiology.Here we present a case of Rosai Dorfman disease of the spine causing lumbo-sacral radiculopathy. CT and MRI revealed lytic expansile lesion in the left half of L4 vertebra mainly involving posterior aspect involving left pedicle and transverse process. Patient underwent surge...
Source: Journal of Clinical Orthopaedics and Trauma - January 25, 2020 Category: Orthopaedics Source Type: research

Apparent Lack of BRAFV600E Derived HLA Class I Presented Neoantigens Hampers Neoplastic Cell Targeting by CD8+ T Cells in Langerhans Cell Histiocytosis
Langerhans Cell Histiocytosis (LCH) is a neoplastic disorder of hematopoietic origin characterized by inflammatory lesions containing clonal histiocytes (LCH-cells) intermixed with various immune cells, including T cells. In 50–60% of LCH-patients, the somatic BRAFV600E driver mutation, which is common in many cancers, is detected in these LCH-cells in an otherwise quiet genomic landscape. Non-synonymous mutations like BRAFV600E can be a source of neoantigens capable of eliciting effective antitumor CD8+ T cell responses. This requires neopeptides to be stably presented by Human Leukocyte Antigen (HLA) class I molecu...
Source: Frontiers in Immunology - January 10, 2020 Category: Allergy & Immunology Source Type: research

TLR7 Agonism Accelerates Disease and Causes a Fatal Myeloproliferative Disorder in NZM 2410 Lupus Mice
Murine models of lupus, both spontaneous and inducible, are valuable instruments to study SLE pathogenesis. Accelerants such as Type I IFN are often used to trigger earlier disease onset. We used a topical TLR7 agonist, previously reported to induce lupus-like disease in WT mice within weeks, to validate this data in C57BL/6j mice, and to test TLR7 agonism as an accelerant in lupus-prone NZM2410 mice. We found that TLR7-stimulated B6 and NZM2410 mice had significantly reduced survival and exhibited profound splenomegaly with significantly reduced B cells (4 vs. 40%), and T cells (8 vs. 31%). Spleen pathology and IHC reveal...
Source: Frontiers in Immunology - January 10, 2020 Category: Allergy & Immunology Source Type: research

Circulating cell-free BRAFV600E during chemotherapy is associated with prognosis of children with Langerhans cell histiocytosis.
PMID: 31919077 [PubMed - as supplied by publisher] (Source: Haematologica)
Source: Haematologica - January 9, 2020 Category: Hematology Authors: Cui L, Zhang L, Ma HH, Wang CJ, Wang D, Lian HY, Li WJ, Zhang Q, Li N, Wang TY, Li ZG, Zhang R Tags: Haematologica Source Type: research

Empirical treatment of massive lymphadenopathy in a child with mixed type histiocytosis in Kenya
Publication date: 4–10 January 2020Source: The Lancet, Volume 395, Issue 10217Author(s): Sarah Muma, Brian Jason Brotherton, Peter Halestrap (Source: The Lancet)
Source: The Lancet - January 3, 2020 Category: General Medicine Source Type: research

Acquired Von Willebrand Syndrome Secondary to Langerhans Cell Histiocytosis
(Source: Indian Journal of Pediatrics)
Source: Indian Journal of Pediatrics - January 3, 2020 Category: Pediatrics Source Type: research

Langerhans cell histiocytosis of the orbit and frontal sinus of the adult woman: A first case report in Poland.
Conclusion: The main purpose of this case report is that LCH should be considered as one of the possible causes of quickly appearing tumor of the orbit in adults. PMID: 31893135 [PubMed] (Source: Surgical Neurology International)
Source: Surgical Neurology International - January 2, 2020 Category: Neurosurgery Tags: Surg Neurol Int Source Type: research

Rosai–Dorfman disease manifesting as epibulbar and orbital tumor: A case report and literature review
Conclusion: This appears to be the first report of RDD manifesting as epibulbar and orbital tumor. Pre-operative diagnosis of RDD remains challenging. When the lesion causes diplopia, surgical resection is the most effective treatment. (Source: Medicine)
Source: Medicine - January 1, 2020 Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research

Congenital Solitary Orbital Juvenile Xanthogranuloma. Report of a Case With Challenging Clinical and Microscopic Presentation
In conclusion, JXG is capable of spontaneous regression, and for this, its management can be conservative. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - January 1, 2020 Category: Pathology Tags: Case Reviews Source Type: research

Combined Erdheim-Chester Disease and Langerhans Cell Histiocytosis in the Lung: A Report of 2 Patients With Overlap Syndrome
We report two cases of ECD-LCH “overlap syndrome” occurring in the lung. In both cases, the diagnoses were supported by typical immunohistochemical patterns, and in both cases, the BRAF V600E mutation was identified by next-generation sequencing and confirmed by droplet digital polymerase chain reaction. In neither case was either diagnosis suspected. The recognition of overlap histiocytoses is important. Although LCH and ECD may differ in treatment and prognosis, in some cases, such as the two patients reported here, they may have the same activating mutations and may be able to be treated with the same target...
Source: Pathology Case Reviews - January 1, 2020 Category: Pathology Tags: Case Reviews Source Type: research

Langerhans Cell Histiocytosis With Solitary Hepatic Involvement in an Adult Patient: Diagnosis, Transplantation, and Recurrence
We describe an unusual case of LCH with solitary hepatic involvement. Histopathologic materials from both native and allograft specimens, including all biopsies, explant hepatectomies, and correlating clinical data accumulated over a 10-year interval, were reviewed. A 46-year-old woman presented with jaundice and pruritus. An initial workup including liver biopsy led to the diagnosis of antimitochondrial antibody–negative primary biliary cholangitis with advanced fibrosis. Within a year, the patient progressed to decompensated cirrhosis requiring transplantation. Her posttransplant course was complicated by frequent ...
Source: Pathology Case Reviews - January 1, 2020 Category: Pathology Tags: Case Reviews Source Type: research

Spinal epidural involvement in adult Langerhans cell histiocytosis (LCH): A case report
We report a case of a 33-year-old man who complained of gait disturbance with weakness in both legs and severe back pain. Diagnoses: A continuous enhancing epidural lesion with cord compression from the T7 to L1 level was detected in magnetic resonance imaging. Laboratory analysis indicated the possibility of spinal infectious disease. We assumed that the lesion could be tuberculous spondylitis. Interventions and outcomes: The patient underwent posterior laminectomy with marginal excision of the epidural mass to relieve cord compression. Pathological examination confirmed the diagnosis of LCH. The 12-month follow-up ...
Source: Medicine - January 1, 2020 Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research

Langerhans Cell Histiocytosis in an Adult: A Discussion of Epidemiology and Treatment Options
In this study, a fibula-free flap was used for subsequent reconstruction. The purpose of the study is 2-fold: to highlight the variability of LCH in both patient symptomatology and demographics, as well as the role of plastic reconstructive surgery in definitive LCH management, particularly in the setting of single system unifocal disease. (Source: Journal of Craniofacial Surgery)
Source: Journal of Craniofacial Surgery - December 27, 2019 Category: Surgery Tags: Brief Clinical Studies Source Type: research

Cytological diagnosis of Rosai & #8211;Dorfman disease: A study of twelve cases with emphasis on diagnostic challenges
Conclusions: FNAC, a simple and cost-effective method with its unique cytology features is the first line of investigation in the diagnosis of Rosai–Dorfman disease. (Source: Journal of Cytology)
Source: Journal of Cytology - December 23, 2019 Category: Cytology Authors: Rallapalli Rajyalakshmi Mohammad Akhtar Yarlagadda Swathi Ranjani Chakravarthi Jeeru Bhaskara Reddy Maddirala Beulah Priscilla Source Type: research

Isolated Langerhans cell histiocytosis in the hypothalamic-pituitary region: a case report
Langerhans cell histiocytosis (LCH) is a rare disease that mainly affects children, but this disease is significantly rarer in patients who are older than 15  years. In this disease, any organ can be involved. ... (Source: BMC Endocrine Disorders)
Source: BMC Endocrine Disorders - December 19, 2019 Category: Endocrinology Authors: Weibin Zhou, Jia Rao and Chengjiang Li Tags: Case report Source Type: research

MR imaging features of orbital Langerhans cell Histiocytosis
To investigate the magnetic resonance imaging (MRI) features of orbital Langerhans cell histiocytosis (LCH) to improve diagnostic accuracy. (Source: BMC Ophthalmology)
Source: BMC Ophthalmology - December 19, 2019 Category: Opthalmology Authors: Chunnan Wu, Kuncheng Li, Yan Hei, Pengyu Lan and Xuetao Mu Tags: Research article Source Type: research

A rare case of non-Langerhans cell histiocytosis/ Erdheim-Chester disease presenting as desquamative gingivitis and oral mucosal erythema
ConclusionsThis is the first report of potential oral mucosal involvement of ECD, with the noted desquamative gingivitis and erythema as its oral presentation. (Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology)
Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology - December 15, 2019 Category: ENT & OMF Source Type: research

An ulcer affecting the gingiva and hard palate: an interesting clinical presentation of langerhans cell histiocytosis
A 37-year-old white female patient was referred for diagnosis of a lesion on alveolar ridge that extended to the hard palate. In reviewing her medical history, the patient reported headaches that led her to have a computed tomography (CT) scan performed. As a result, fibrous dysplasia of the ethmoidal sinus was diagnosed. Physical examination revealed an ulcerated lesion, with irregular contours, erythroplasic areas, partially covered by fibrin membrane, and without pain. Radiographic examination revealed the destructive character of the lesion, evidenced by the presence of bone loss between teeth 15 and 16. (Source: Oral ...
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - December 15, 2019 Category: ENT & OMF Authors: JEFFERSON DA ROCHA TENORIO, MANUELA COLBECK GON ÇALVES CARVALHO, MARCOS CUSTÓDIO, FÁBIO DAUMAS NUNES, KAREM LÓPEZ ORTEGA Source Type: research

Langerhans cell histiocytosis
(LCH) is a rare condition of uncertain etiology resulting from a clonal proliferation of Langerhans cells, which mainly affects children. In March 2018, parents of a 1-year-old male patient reported a volume increasing on the right side of the child's jaw. The imaging tests revealed a lytic lesion with aggressive aspect in the mandible. In view of this, it was decided to perform an incisional biopsy of the affected area and subsequent histopathologic examination. The histologic slices showed a diffuse infiltration of large monocyte cell resembling histiocytes, numerous plasma cells, lymphocytes, and eosinophils. (Source: ...
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - December 15, 2019 Category: ENT & OMF Authors: R ÚBIA DA ROCHA VIEIRA, KARINE RASERA, PAULA LUCE BOHRER Source Type: research

Oral manifestations in langerhans cell histiocytosis. a case report
We present a case of a 68-year-old woman diagnosed as having multisystem Langerhans cell histiocytosis (LCH) 8 years ago. She was treated with chemotherapy last year because systemic affectation; the patient has complained about intense pain and swelling of gums beginning 2 years ago. Intraoral examination reveals poor oral hygiene, gingival enlargement, and multiple necrotic ulcerations covered by pseudomembrane involving the upper buccal gingiva, the intercanine region, and the lower buccal and lingual gingiva of premolars, canines, and incisives. (Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics)
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - December 15, 2019 Category: ENT & OMF Authors: PILAR GANDARA-VILA, CATALINA BARBA-MONTERO, JOSE MANUEL SUAREZ-PE ÑARANDA, JOSE MANUEL SOMOZA-MARTÍN, ABEL GARCIA-GARCIA, ANDRES BLANCO-CARRION, MARIO PEREZ-SAYANS Source Type: research

Oral langerhans cell histiocytosis: periradicular and peri-implant involvement
Langerhans cell histiocytosis is an uncommon disease of unknown pathogenesis characterized by the proliferation of Langerhans cells. Oral involvement can produce lesions that mimic periodontal and periradicular inflammatory lesions, but peri-implant lesions have been rarely described. A 40-year-old man diagnosed as having cutaneous involvement by Langerhans cell histiocytosis 4 years before was referred for evaluation of progressive alveolar bone loss lasting 8 months. Panoramic radiography showed extensive bone resorption in all 4 quadrants but especially in the anterior mandible and left posterior maxilla. (Source: Oral ...
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - December 15, 2019 Category: ENT & OMF Authors: THAIS SOARES DA SILVA, PAULO JOS É MEDEIROS, JULIA FRANCIS DA SILVA PEREIRA, TERESA CRISTINA RIBEIRO BARTHOLOMEU DOS SANTOS, FÁBIO RAMOA PIRES, IOLANDA ZANOTELI LEMOS, RAFFAEL MARON VIEIRA DE NORONHA Source Type: research

Histiocytosis: case report
Histiocytosis is part of histiocyte-related proliferative diseases. The term histiocyte involves all types of macrophages and other dendritic cells (antigen-presenting cells: dermal dendrocytes, Langerhans cells (CL), and indeterminate cells). An 11-year-old male patient presented with an initial complaint of dental pain. Intraoral examination revealed exophytic and ulcerated lesions associated with the left maxillary first and second premolar and with left mandibular first and second molar with a time-course evolution of 2 months. (Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics)
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - December 15, 2019 Category: ENT & OMF Authors: WILKELLY ALVES DE LIMA, PABLYANNE TEREZA LOUZADA GUEDES, GLAUCE GUIMAR ÃES PEREIRA, LUCAS CARVALHAES PERES, ANA CAROLINA SANTIAGO DA SILVA, FLAVIA SIROTHEAU CORREA PONTES, HELDER ANTONIO REBELO PONTES Source Type: research

A rare case of non-Langerhans cell histiocytosis/ Erdheim-Chester disease presenting as desquamative gingivitis and oral mucosal erythema
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by BRAFV600 E mutations in greater than 50% of patients; multisystemic involvement, such as skin (xanthelasma); heart (pericardial effusion); central nervous system (brain-stem lesions); and bone lesions. ECD has a distinct histopathology and immunochemistry profile, which is negative for CD1 a, S100, and Langerin. Craniofacial involvement has been reported in less than 10 cases and described as multiple, solitary, well-defined radiopaque/lucent lesions of the jaws. (Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics)
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - December 15, 2019 Category: ENT & OMF Authors: Muhammad Ali Shazib, Herv é Yves Sroussi Tags: Case Report Presentations: The following high-quality case report abstracts were selected for oral presentations. Source Type: research

Erdheim-Chester disease presenting with chorea and mimicking IgG4-related disorder
Consider ECD in patients with multisystemic disease with neurologic manifestations. IgG4-related disorder is a possible mimic of ECD, and ECD and Langerhans cell histiocytosis may occur concomitantly with unusual neurologic presentations, including chorea. (Source: Neurology Clinical Practice)
Source: Neurology Clinical Practice - December 9, 2019 Category: Neurology Authors: Miron, G., Karni, A., Faust-Soher, A., Giladi, N., Alroy, H., Gadoth, A. Tags: All Clinical Neurology, Hematologic, Chorea Case Source Type: research

Retrospective study of Langerhans cell histiocytosis in ear, nose and neck
ConclusionLCH mostly occurs in children. In head and neck it affects principally the mastoid part of the temporal bone. Treatments include surgery, chemotherapy, and irradiation. Most patients enjoy good prognoses. LCH accompanied by TCS is rare and increases the difficulty of diagnosis; molecular data aid in TCS identification. (Source: American Journal of Otolaryngology)
Source: American Journal of Otolaryngology - December 7, 2019 Category: Endocrinology Source Type: research

Solitary Bone Langerhans Cell Histiocytosis Demonstrated on Multimodality Imaging in an Adult
Multimodality imaging was performed on a 53-year-old woman who was suspected with malignant bone tumor in her left femoral midshaft on radiograph. An MRI demonstrated the lesion showed hypointensity on T1-weighted and high intensity on T2-weighted fat-saturated images. It was more extensive on MRI than that on radiograph, especially in bone marrow and parosteal soft tissue. The lesion accumulated radiotracer on bone scan and 18F-FDG PET/CT. Finally, a Langerhans cell histiocytosis was confirmed after surgery. The case cautions us Langerhans cell histiocytosis should be in the differential diagnosis spectrum in an adult wit...
Source: Clinical Nuclear Medicine - December 7, 2019 Category: Nuclear Medicine Tags: Interesting Images Source Type: research

Spectrum of 18F-FDG Uptake in Bilateral Lung Parenchymal Diseases on PET/CT
Bilateral lung parenchymal involvement is seen in infective as well as noninfective conditions, appearing as focal or diffuse lung disease. PET/CT with FDG helps in characterization (increased glucose utilization is seen by both inflammatory and neoplastic cells). In this article, we describe the spectrum of patterns of FDG uptake and associated CT changes involving bilateral lung parenchyma. Benign conditions described are aspiration pneumonia; pulmonary toxicity by bleomycin; infections, namely, sarcoidosis, miliary pulmonary tuberculosis, and pulmonary nocardiosis; and inflammatory conditions such as pulmonary Langerhan...
Source: Clinical Nuclear Medicine - December 7, 2019 Category: Nuclear Medicine Tags: Interesting Images Source Type: research

Erdheim-Chester Disease: a Concise Review
This report provides an overview of the current knowledge of molecular characterization, clinical description, and treatment of Erdheim-Chester disease (ECD), a multi-systemic adult histiocytosis of the L group.Recent FindingsThe recent identification of several MAPK mutations in histiocytes of ECD lesions. Leading to targeted therapies.SummaryThe discovery of theBRAFV600E mutation in ECD lesions followed by several other kinase mutations in the MAPK pathway has revolutionized our understanding of the disease pathogenesis and led to trials with targeted therapies that demonstrated robust efficacy. (Source: Current Rheumatology Reports)
Source: Current Rheumatology Reports - December 5, 2019 Category: Rheumatology Source Type: research

Neuroimaging features of CNS histiocytosis syndromes
Histiocytosis syndromes (HS) are group of heterogeneous disorders characterized by abnormal accumulation and infiltration of histiocytes, cells derived from hematopoietic cells of monocyte/macrophage lineage. Overall these disorders are rare. When they do occur they involve many organ systems including the central nervous system (CNS). While imaging findings can provide important clues, diagnosis of this disorder is challenging and definitive diagnosis often necessitates pathologic examination. In this review, we describe imaging features of HS involving the CNS, with the aim to increase our understanding of these disorder...
Source: Clinical Imaging - December 5, 2019 Category: Radiology Authors: Yuting Wang, Sandra Camelo-Piragua, Asif Abdullah, Mohannad Ibrahim, Hemant A. Parmar Tags: Neuroradiology Source Type: research

Adult Onset Langerhans Cell Histiocytosis Diagnosed With Xanthoma-like Plaque
Publication date: Available online 2 December 2019Source: Actas Dermo-Sifiliográficas (English Edition)Author(s): E. Boyuk, I. Bulur, F. Canaz, O. Cilingir (Source: Actas Dermo-Sifiliograficas)
Source: Actas Dermo-Sifiliograficas - December 3, 2019 Category: Dermatology Source Type: research

Edema Surrounding Benign Tumors and Tumor-Like Lesions.
Conclusion: Bone marrow and soft tissue edema are a common finding associated with benign bone tumors and tumor-like diseases, and they are frequently detected in Langerhans cell histiocytosis, osteoblastoma, osteoid osteoma, and chondroblastoma. PMID: 31781646 [PubMed - in process] (Source: Biomed Res)
Source: Biomed Res - December 1, 2019 Category: Research Authors: Gao S, Zhou R, Xu Q, Chen H Tags: Biomed Res Int Source Type: research

Indeterminate cell histiocytosis in a patient with systemic lupus erythematosus and antiphospholipid antibody syndrome: an unusual association.
Abstract Indeterminate cell histiocytosis (ICH) is an extremely rare clonal proliferative disorder of dendritic cells which presents with skin lesions in the majority of cases. Although extra-cutaneous manifestations are very rare, ICH may involve the mucosa, cornea, and conjunctiva as well as the visceral organs. Since the clinical appearance of cutaneous lesions of ICH is not distinctive, it is diagnosed with histopathological and immunohistochemical findings after clinical suspicion. Herein, we report a 27-year-old man with a two-year history of asymptomatic reddish papules and papulonecrotic lesions on his fac...
Source: Lupus - December 1, 2019 Category: Rheumatology Authors: Akdogan N, Demircan C, Cagri Bolek E, Gokoz O, Karaduman A Tags: Lupus Source Type: research

Nationwide retrospective review of hematopoietic stem cell transplantation in children with refractory Langerhans cell histiocytosis.
/Oncology Abstract The efficacy of and indications for hematopoietic stem cell transplantation (HSCT) in pediatric Langerhans cell histiocytosis (LCH) remain undetermined. This retrospective study analyzed 30 children with refractory LCH who underwent HSCT in Japan between 1996 and 2014. Eleven patients received a myeloablative conditioning (MAC) regimen, while 19 patients received a reduced-intensity conditioning (RIC) regimen. Among the 26 patients with complete data, 23 patients had risk organ (RO) involvement during clinical course. Disease status at HSCT was no active disease (NAD) (4), active disease-regress...
Source: International Journal of Hematology - November 22, 2019 Category: Hematology Authors: Kudo K, Maeda M, Suzuki N, Kanegane H, Ohga S, Ishii E, Shioda Y, Imamura T, Imashuku S, Tsunematsu Y, Endo M, Shimada A, Koga Y, Hashii Y, Noguchi M, Inoue M, Tabuchi K, Morimoto A, Histiocytosis study group of the Japanese Society of Pediatric Hematolog Tags: Int J Hematol Source Type: research

Severe Langerhans Cell Histiocytosis with Pulmonary Involvement
Conclusion: The most prominent feature of these two patients was severe respiratory involvement at the time of diagnosis. It should be kept in mind that lung involvement may be the first finding in patients with LCH and can appear in very young ages. BRAF mutation should be investigated in severe cases with LCH. (Source: European Respiratory Journal)
Source: European Respiratory Journal - November 21, 2019 Category: Respiratory Medicine Authors: Cimen, O., Emiralioglu, N., Gharibzadehhızal, M., Bayindir, Y., Eryilmazpolat, S., Ademhan, D., Oguz, B., Orhan, D., Ozcan, N., Aydın, B., Soyer, T., Yalcın, E., Dogru, D., Ozcelik, U., Kiper, N. Tags: Paediatric bronchology Source Type: research

Psychological abnormalities are commonly present in adult patients with pulmonary Langerhans cell histiocytosis
Conclusions: Psychological abnormalities commonly co-exist in adult PLCH patients and should be considered while making their management decisions. (Source: European Respiratory Journal)
Source: European Respiratory Journal - November 21, 2019 Category: Respiratory Medicine Authors: Tazi, A., Bugnet, E., Gupta, N., Lorillon, G., Arbabzadeh-Bouchez, S., Lepine, J.-P., Chevret, S. Tags: Rare ILD/DPLD Source Type: research

Body mass index predicts survival in patients with pulmonary hypertension in chronic lung disease
Conclusion: BMI is a common marker for predicting survival in patients with PH across various lung disorders. (Source: European Respiratory Journal)
Source: European Respiratory Journal - November 21, 2019 Category: Respiratory Medicine Authors: Nishiyama, O., Yoshikawa, K., Saeki, S., Yamazaki, R., Sano, H., Iwanaga, T., Tohda, Y. Tags: Pulmonary hypertension Source Type: research