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    10475 results match your criteria Langerhans Cell Histiocytosis

    1 OF 210

    Cholesteatoma as a complication of Langerhans Cell Histiocytosis of the temporal bone: A nationwide cross-sectional analysis.
    Int J Pediatr Otorhinolaryngol 2017 Sep 22;100:66-70. Epub 2017 Jun 22.
    Department of Pediatric Otolaryngology, Floating Hospital for Children at Tufts Medical Center, 800 Washington St. Boston, MA 02111, United States. Electronic address:
    Objective: To determine if patients with Langerhans Cell Histiocytosis (LCH) of the temporal bone have a higher risk of developing cholesteatoma.

    Methods: Review of literature and cross-sectional weighted analysis of patients under 19 with a diagnosis of LCH from the National Inpatient Sample (NIS) and Kids' Inpatient Database (KID) from 2000 to 2013. ICD-9 codes and demographics were analyzed; pairwise comparisons and multivariate analyses were performed. Read More

    Hematopoietic Tumors Primarily Presenting in Bone.
    Surg Pathol Clin 2017 Sep 27;10(3):675-691. Epub 2017 Jun 27.
    Department of Pathology, Leiden University Medical Center, PO Box 9600, L1-Q, Leiden 2300 RC, The Netherlands.
    Hematologic neoplasms that primarily present in bone are rare; this article describes the most common examples of hematologic tumors primarily presenting in bone, including plasma cell myeloma, solitary plasmacytoma of bone, primary non-Hodgkin lymphoma of bone, acute lymphoblastic leukemia/lymphoma, and Langerhans cell histiocytosis. The macroscopic and microscopic features, differential diagnosis, diagnostic workup, and prognosis of all these different entities are discussed, with special emphasis on common differential diagnosis. Read More

    Differential diagnosis of granulomatous lung disease: clues and pitfalls: Number 4 in the Series "Pathology for the clinician" Edited by Peter Dorfmüller and Alberto Cavazza.
    Eur Respir Rev 2017 Sep 9;26(145). Epub 2017 Aug 9.
    Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany
    Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Read More

    [Eosinophilic granuloma of the parietal bone of an adult patient with BRAF mutation].
    Arkh Patol 2017 ;79(4):33-39
    Academician B.V. Petrovsky Russian Research Center of Surgery, Moscow, Russia.
    The paper describes a case of eosinophilic granuloma of the parietal bone in a 32-year-old man. Histological examination revealed a large number of bean-shaped Langerhans cell histiocytes with lobed nuclei and nuclear grooves. The histiocytes alternated with the foci of obvious eosinophilic infiltration and with eosinophilic microabscesses. Read More

    Cardiorespiratory fitness and physical function in children with cancer from diagnosis throughout treatment.
    BMJ Open Sport Exerc Med 2017 12;3(1):e000179. Epub 2017 May 12.
    Department of Sports Medicine, Norwegian School of Sport Sciences, Oslo, Norway.
    Background: Children with cancer experience severe reductions in physical fitness and functionality during and following intensive treatment. This may negatively impact their quality of life.

    Purpose: To describe the physical capacity and functionality of children with cancer during and after treatment as well as the feasibility of physical activity intervention in the Rehabilitation including Social and Physical activity and Education in Children and Teenagers with Cancer study. Read More

    Case Report of a Hypobaric Chamber Fitness to Fly Test in a Child With Severe Cystic Lung Disease.
    Pediatrics 2017 Jul 8;140(1). Epub 2017 Jun 8.
    Pediatric Respiratory Department, Starship Children's Hospital, Auckland, New Zealand; and
    Patients with severe cystic lung disease are considered to be at risk for cyst rupture during air travel because of the possibility of increase in cyst size and impaired equilibration of pressure between the cysts and other parts of the lung. This may have clinically devastating consequences for the patient but may also result in significant costs for emergency alteration of flight schedule. We report the use of a hypobaric chamber to simulate cabin pressure changes encountered on a commercial flight to assess the safety to fly of a child with severe cystic lung disease secondary to Langerhans cell histiocytosis. Read More

    Novel activating BRAF fusion identifies a recurrent alternative mechanism for ERK activation in pediatric Langerhans cell histiocytosis.
    Pediatr Blood Cancer 2017 Jul 27. Epub 2017 Jul 27.
    Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, New York, New York.
    Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm characterized by constitutive activation of extracellular signal-regulated kinase (ERK). Genomic characterization has identified activating point mutations including mutually exclusive BRAFV600E and activating MAP2K1 mutations to be responsible for ERK activation in a majority of pediatric LCH patients. Here, we report the discovery of a novel BRAF kinase fusion, PACSIN2-BRAF, in a child with multisystem LCH. Read More

    Role of (18)F-FDG PET/CT in patients affected by Langerhans cell histiocytosis.
    Jpn J Radiol 2017 Jul 26. Epub 2017 Jul 26.
    Nuclear Medicine, University of Brescia and Spedali Civili Brescia, Brescia, Italy.
    Purpose: Langerhans cell histiocytosis (LCH) is a rare hematological disorder for which the utility of(18)F-FDG PET/CT is unclear. Our aim was to explore the metabolic features of LCH and the possible role of(18)F-FDG PET/CT in LCH evaluation.

    Materials And Methods: We found 17 patients with histologically proven LCH who underwent 17(18)F-FDG PET/CT scans for staging and 42 scans for restaging/follow-up purposes. Read More

    Lung Function in Pregnancy in Langerhans Cell Histiocytosis.
    Adv Exp Med Biol 2017 Jul 26. Epub 2017 Jul 26.
    Third Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, 26 Płocka Street, 01-138, Warsaw, Poland.
    Pulmonary Langerhans cell histiocytosis (LCH) is a rare disease, affecting usually young people. The course of the disease is variable. In some pulmonary LCH patients a severe lung destruction and progression in spite of chemotherapy is observed, but in others just a cessation of smoking induces a regression of the disease. Read More

    Unique Case of Hearing Recovery After Otic Capsule Destruction and Complete Sensorineural Hearing Loss Caused by Langerhans Cell Histiocytosis.
    Otol Neurotol 2017 Sep;38(8):1129-1132
    Division of Pediatric Otolaryngology-Head and Neck Surgery, British Columbia Children's Hospital, Vancouver, British Columbia, Canada.
    : A 14-year-old woman presented with right-sided otologic and vestibular symptoms after presenting with hormonal disturbances earlier that year. Imaging showed a gross destruction of the temporal bone, mastoid air cells, and external acoustic meatus with invasion into the otic capsule. The patient experienced complete sensorineural hearing loss in the right ear. Read More

    Molluscum Contagiosum-Like Presentation of Langerhans Cell Histiocytosis: A Case and Review.
    Pediatr Dermatol 2017 Jul 21. Epub 2017 Jul 21.
    Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada.
    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. Read More

    [Diagnostic utility of tyrosine hydroxylase in peripheral neuroblastic tumors].
    Zhonghua Bing Li Xue Za Zhi 2017 Jul;46(7):471-475
    Department of Pathology, Children Hospital, Capital Medical University, Beijing 100045, China.
    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). Tissue slides of these 1 024 cases were stained with TH and other common antibodies to evaluate whether TH was useful to identify pNT. Read More

    Assessment of BRAF(V600E) mutation in pulmonary Langerhans cell histiocytosis in tissue biopsies and bronchoalveolar lavages by droplet digital polymerase chain reaction.
    Virchows Arch 2017 Jul 15. Epub 2017 Jul 15.
    Laboratory of Tumor Biology and Tumor Biobank, CHU Bordeaux, Pessac, F-33604, Bordeaux, France.
    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). Read More

    Eosinophilic granuloma at the cerebellopontine angle in an adult; a rare case report and literature review.
    Int J Surg Case Rep 2017 29;37:183-185. Epub 2017 Jun 29.
    Pathology Department, Shiraz University of Medical Sciences, Shiraz, Iran.
    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. Read More

    Histiocytic and dendritic cell neoplasms: what have we learnt by studying 67 cases.
    Virchows Arch 2017 Jul 10. Epub 2017 Jul 10.
    Institute of Pathology and Neuropathology and Comprehensive Cancer Center, University Hospital Tübingen, Tübingen, Germany.
    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. Read More

    Delayed Treatment Response in a Neonate with Multisystem Langerhans Cell Histiocytosis Case report and review of literature.
    Sultan Qaboos Univ Med J 2017 May 20;17(2):e225-e228. Epub 2017 Jun 20.
    Department of Pediatrics, Chacha Nehru Bal Chikitsalya, New Delhi, India.
    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. Read More

    Erdheim-Chester Disease: Case Report with Aggressive Multisystem Manifestations and Review of the Literature.
    Case Rep Oncol 2017 May-Aug;10(2):501-507. Epub 2017 Jun 14.
    Department of Central Military Laboratory and Blood Bank, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
    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. Read More

    Current understanding and management of pulmonary Langerhans cell histiocytosis.
    Thorax 2017 Jul 8. Epub 2017 Jul 8.
    Department of Pulmonary Medicine, Saint-Louis Hospital, National Reference Center for Langerhans Cell Histiocytosis, University Paris Diderot, Sorbonne Paris Cite, Inserm UMR-1153 (CRESS), Biostatistics and Clinical Epidemiology Research Team (ECSTRA), Paris, Ile-de-France, France.
    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). Read More

    Pediatric Langerhans cell histiocytosis of the lateral skull base.
    Int J Pediatr Otorhinolaryngol 2017 Aug 15;99:135-140. Epub 2017 Jun 15.
    Department of Otolaryngology - Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA. Electronic address:
    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. Read More

    Tocilizumab in patients with multisystem Erdheim-Chester disease.
    Oncoimmunology 2017 20;6(6):e1318237. Epub 2017 Apr 20.
    Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy.
    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. Read More

    High prevalence of myeloid neoplasms in adults with non-Langerhans cell histiocytosis.
    Blood 2017 Jul 5. Epub 2017 Jul 5.
    Centre de Reference des Histiocytoses, Hopital de la Pitie-Salpetriere, Assistance Publique - Hopitaux de Paris, Universite Pierre et Marie Curie, Paris, France;
    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. Read More

    New somatic BRAF splicing mutation in Langerhans cell histiocytosis.
    Mol Cancer 2017 Jul 6;16(1):115. Epub 2017 Jul 6.
    EA4340, Versailles SQY University, Paris-Saclay University, Boulogne, France.
    Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with constitutive activation of the MAPKinase RAS-RAF-MEK-ERK cell signaling pathway. We analyzed 9 LCH cases without BRAF (V600) and MAP2K1 mutations by whole exome sequencing. We identified a new somatic BRAF splicing mutation in 2 cases. Read More

    Identification and Targeting of Kinase Alterations in Histiocytic Neoplasms.
    Hematol Oncol Clin North Am 2017 Aug 17;31(4):705-719. Epub 2017 May 17.
    Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA. Electronic address:
    Histiocytic disorders represent clonal disorders of cells believed to be derived from the monocyte, macrophage, and/or dendritic cell lineage presenting with a range of manifestations. Although their nature as clonal versus inflammatory nonclonal conditions have long been debated, recent studies identified numerous somatic mutations that activate mitogen-activated protein kinase signaling in clinically and histologically diverse forms of histiocytosis. Clinical trials and case series have revealed that targeting aberrant kinase signaling using BRAF and/or MEK inhibitors may be effective. Read More

    Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai-Dorfman disease.
    Mod Pathol 2017 Jun 30. Epub 2017 Jun 30.
    Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
    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. Read More

    Erdheim-Chester Disease Involving the Central Nervous System with the Unique Appearance of a Coated Vertebral Artery.
    NMC Case Rep J 2016 Oct 29;3(4):125-128. Epub 2016 Aug 29.
    Department of Neurosurgery, Sapporo Medical University School of Medicine, Hokkaido, Japan.
    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. Read More

    A Case of Refractory Langerhans Cell Histiocytosis Complicated with Hemophagocytic Lymphohistiocytosis Rescued by Cord Blood Transplantation with Reduced-intensity Conditioning.
    Acta Med Okayama 2017 Jun;71(3):249-254
    Department of Pediatrics, Okayama University Hospital, Okayama 700-8558, Japan.
     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. Read More

    Lung ultrasound has limited diagnostic value in rare cystic lung diseases: a cross-sectional study.
    Eur Clin Respir J 2017 1;4(1):1330111. Epub 2017 Jan 1.
    Department of Respiratory Medicine, Odense University Hospital, Odense C, Denmark.
    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). Read More

    The outcome of eosinophilic granuloma involving unilateral atlantoaxial joint: A case report and literature review.
    Medicine (Baltimore) 2017 Jun;96(25):e7197
    aDepartment of Orthopedics, Union Hospital, Tongji Medical College of Huazhong University of Science and Technology, Wuhan, Hubei bDepartment of Ophthalmology, Shengjing Hospital of China Medical University, Shenyang, Liaoning cDepartment of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
    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. Read More

    A rare case of oral multisystem Langerhans cell histiocytosis.
    J Clin Exp Dent 2017 Jun 1;9(6):e820-e824. Epub 2017 Jun 1.
    Oral Surgery Unit of George Eastman Hospital, Umberto I Teaching Hospital, Rome, Italy.
    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. Read More

    Chronic Recurrent Multifocal Osteomyelitis: A Case Report with Atypical Presentation.
    J Orthop Case Rep 2017 Jan-Feb;7(1):75-78
    Department of Orthopaedics, Hospital Prof. Doutor Fernando Fonseca, Estrada IC19, 2720-276 Amadora, Portugal.
    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. Read More

    Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion.
    Case Rep Pathol 2017 24;2017:9842605. Epub 2017 May 24.
    Department of Cellular Pathology, Royal Devon and Exeter NHS Foundation Trust, Old Pathology Building, Church Lane, Exeter EX2 5DW, UK.
    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. Read More

    Association between Ischemic Stroke and Erdheim-Chester Disease: A Case Report and Review of Literature.
    J Stroke Cerebrovasc Dis 2017 Aug 13;26(8):e153-e155. Epub 2017 Jun 13.
    Neurology Department, Meaux General Hospital, Meaux, France.
    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. Read More

    Cyclin D1 is Expressed in Neoplastic Cells of Langerhans Cell Histiocytosis but Not Reactive Langerhans Cell Proliferations.
    Am J Surg Pathol 2017 Jun 15. Epub 2017 Jun 15.
    Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA.
    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. Read More

    A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults.
    Cancer 2017 Jun 16. Epub 2017 Jun 16.
    Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.
    Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations, and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition. Read More

    Erdheim-Chester Disease presenting with histiocytic colitis and cytokine storm.
    J Gastrointestin Liver Dis 2017 Jun;26(2):183-187
    Division of Gastroenterology, Department of Medicine Washington University School of Medicine, St. Louis, MO, USA.
    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. Read More

    Metastatic pancreatic carcinoma masquerading as cystic lung disease: a rare presentation.
    Respirol Case Rep 2017 Sep 11;5(5):e00246. Epub 2017 Jun 11.
    Department of Respiratory MedicineSir Charles Gairdner HospitalPerthAustralia.
    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. Read More

    A Case of Pulmonary Langerhans Cell Sarcoma Simultaneously Diagnosed with Cutaneous Langerhans Cell Histiocytosis Studied by Whole-Exome Sequencing.
    Acta Haematol 2017 Jun 15;138(1):24-30. Epub 2017 Jun 15.
    Department of Thoracic and Cardiovascular Surgery, Chungbuk National University College of Medicine, Cheongju, Republic of Korea.
    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. Read More

    Congenital Syphilis of Bone: A Potential Mimicker of Childhood Histiocytoses.
    Am J Surg Pathol 2017 Sep;41(9):1283-1289
    *Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH †Pitts Radiology ‡Pediatric Associates, PA, Columbia, SC.
    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. Read More

    Erdheim-Chester Disease Presenting with Secondary Hypertension as a Result of Bilateral, Proximal Renal Artery Stenosis: A Case Report.
    Case Rep Nephrol Dial 2017 May-Aug;7(2):55-62. Epub 2017 May 23.
    University of California, Los Angeles, California, USA.
    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. Read More

    [Clinical features and prognosis of Langerhans cell histiocytosis in children: an analysis of 34 cases].
    Zhongguo Dang Dai Er Ke Za Zhi 2017 Jun;19(6):627-631
    Department of Pediatric Hematology and Oncology, Chengdu Women and Children's Central Hospital, Chengdu 610091, China.
    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. Read More

    PET/MR in the Assessment of Pediatric Histiocytoses: A Comparison to PET/CT.
    Clin Nucl Med 2017 Aug;42(8):582-588
    From the *Department of Pediatric Radiology, Texas Children's Hospital, Houston; and †Section of Hematology/Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, TX.
    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. Read More

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