Search our Database of Scientific Publications and Authors

I’m looking for a

    10512 results match your criteria Langerhans Cell Histiocytosis

    1 OF 211

    A rare case of CD1a-negative Langerhans cell histiocytosis of the central nervous system in a child.
    Clin Case Rep 2017 Oct 1;5(10):1664-1667. Epub 2017 Sep 1.
    Cook Children's Medical CenterFort WorthTexas.
    Langerhans cell histiocytosis is a dendritic cell disorder with a wide spectrum of severity and presentations. Histopathology typically demonstrates a proliferation of Langerhans cells and a lymphohistiocytic inflammatory infiltrate with eosinophils. The diagnosis is supported by immunohistochemistry with the cell markers S100, CD1a, CD68, and Langerin [Blood, 126, 2015, 26 and N Engl J Med, 331, 1994, 154]. Read More

    Atlantoaxial Langerhans cell histiocytosis radiographic characteristics and corresponding prognosis analysis.
    J Craniovertebr Junction Spine 2017 Jul-Sep;8(3):199-204
    Department of Orthopaedics, Peking University Third Hospital, Beijing, PR China.
    Background: Langerhans cell histiocytosis (LCH) may affect atlas and axis, and there were very few published cases describing a characteristic of LCH of atlantoaxial.

    Objective: The objective of the study is to investigate the image manifestations of atlantoaxial LCH to improve the in-depth comprehension on it.

    Materials And Methods: A retrospective study was done of computed tomography (CT) and magnetic resonance imaging in atlas and axis and prognosis was analyzed. Read More

    Occult Langerhans Cell Histiocytosis in Clear Cell Renal Cell Carcinoma.
    Int J Surg Pathol 2017 Oct 1:1066896917735171. Epub 2017 Oct 1.
    1 Yeungnam University, Daegu, South Korea.
    Langerhans cell histiocytosis is a rare disease that is characterized by a localized or systemic proliferation of Langerhans dendritic cells and a wide spectrum of clinical presentations. We experienced an unusual case of occult Langerhans cell histiocytosis associated with clear cell renal cell carcinoma. A 62-year-old man underwent a partial nephrectomy for left renal mass. Read More

    Langerhans Cell Histiocytosis of the Thoracic Spine in an Adult.
    Korean J Spine 2017 Sep 30;14(3):109-111. Epub 2017 Sep 30.
    Department of Neurosurgery, Ewha Womans University College of Medicine, Seoul, Korea.
    We report a case of a 45-year-old man with a complaint of both leg weakness and hypoesthesia. Radiological evaluation revealed an osteolytic lesion of the ninth thoracic vertebra. The patient underwent posterior corpectomy with total excision of the tumor, mesh cage insertion with posterior screw fixation and subsequent radiotherapy. Read More

    Vulvar lesions in an 8-year-old girl: cutaneous manifestations of multisystem Langerhans cell histiocytosis.
    J Pediatr Adolesc Gynecol 2017 Oct 6. Epub 2017 Oct 6.
    Department of Obstetrics, Gynecology & Reproductive Sciences, Yale University School of Medicine, New Haven, CT.
    Background: Langerhans cell histiocytosis (LCH) is a rare localized or systemic disease characterized by proliferation of myeloid-derived dendritic cells. Vulvar lesions may be the herald symptom of LCH and may mimic other cutaneous lesions. Prognosis varies widely based on the extent and spread of disease. Read More

    LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY.
    Rev Paul Pediatr 2017 Jan-Mar;35(1):115-119
    Ataturk Research and Training Hospital, Esmirna, Turquia.
    Objective: To report a case of late-onset self-healing Langerhans cell histiocytosis.

    Case Description: A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later. Read More

    Langerhans Cell Histiocytosis Involving Both Jaws in an Adult.
    J Coll Physicians Surg Pak 2017 Sep;27(9):S89-S91
    Department of Pathology, Dow International Medical College, Dow University of Health Sciences, Ojha Campus, Karachi.
    Langerhans cell histiocytosis (LCH) is the latest terminology for a disorder of reticulo-endothelial system, previously known as histiocytosis X, and marked by aberrant proliferation of bone marrow derived Langerhans cells with variable inflammatory infiltrate including neutrophils, lymphocytes, plasma cells, eosinophils, and multinucleated giant cells. Although rare, the disorder frequently inflicts children with peak incidence recorded in 2-4 years age group. LCH is rare in adults. Read More

    New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.
    Eur Respir Rev 2017 Sep 27;26(145). Epub 2017 Sep 27.
    U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, MultiMedica IRCCS, Milan, Italy
    Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. Read More

    Congenital self-healing reticulohistiocytosis with spontaneous regression.
    An Bras Dermatol 2017 Jul-Aug;92(4):553-555
    Department of Dermatology, Shandong Provincial Hospital of Dermatology and Venereology, Shandong University, Jinan, Shandong, China.
    Congenital self-healing reticulohistiocytosis is a rare, benign, self-limiting variant of Langerhans cell histiocytosis (LCH). LCH encompasses a group of idiopathic disorders characterized by the clonal proliferation of Langerhans cells. Congenital self-healing reticulohistiocytosis typically appears at birth or in the neonatal period as isolated cutaneous lesions, often appearing as multiple crusted papules with no systemic findings. Read More

    Langerhans' cell histiocytosis with neurological injuries diagnosed from a single cutaneous lesion.
    An Bras Dermatol 2017 Jul-Aug;92(4):540-542
    Dermatology Service, Hospital Regional de Presidente Prudente, Presidente Prudente, SP, Brazil.
    Histiocytoses are rare diseases caused by the proliferation of histiocytes. The pathogenesis remains unknown and the highest incidence occurs in pediatric patients. The clinical presentations can be varied, in multiple organs and systems, and the skin lesions are not always present. Read More

    Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: Current views and new vistas.
    Pediatr Blood Cancer 2017 Sep 25. Epub 2017 Sep 25.
    Department of Pediatrics, Texas Children's Cancer and Hematology Centers and Baylor College of Medicine, Houston, Texas.
    Central nervous system (CNS) involvement in Langerhans cell histiocytosis (LCH) can include mass lesions of the hypothalamic pituitary axis, choroid plexus, cerebrum, and cerebellum or magnetic resonance imaging (MRI) signal abnormalities of the cerebellum, pons, and basal ganglia. The term neurodegenerative (ND) CNS-LCH has been given to the MRI signal abnormalities and neurologic dysfunction, although initially patients may have no clinical symptoms. Standardized evaluations to better understand the natural history and response to therapy are needed. Read More

    Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report.
    J Med Case Rep 2017 Sep 25;11(1):272. Epub 2017 Sep 25.
    Lebanese University, Faculty of Medical Sciences, Hadath, Lebanon.
    Background: Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects - by order of decreasing frequency - the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adults. Read More

    Two cases of benign fibrous histiocytomas (dermatofibromas) associated with Langerhans cell histiocytosis.
    Histopathology 2017 Sep 23. Epub 2017 Sep 23.
    Department of Dermatopathology, St. John's Institute of Dermatology, London, Guy's and St. Thomas' Hospitals NHS Foundation Trust, London, United Kingdom.
    We report two cases of fibrous histiocytoma (FH) associated with discrete nodular aggregates of Langerhans cells (LCs) resembling Langerhans cell histiocytosis (LCH). In addition, the LCs showed positivity for BRAF V600E immunohistochemistry, a finding reported in neoplastic but not reactive LCs (1) . To our knowledge, these cases represent the first published association of FH and LCH. Read More

    Demodicosis in two patients with a previous history of Langerhans cell histiocytosis.
    Pediatr Dermatol 2017 Sep 21. Epub 2017 Sep 21.
    Department of Dermatology, Hospital Sant Joan de Déu, Barcelona, Spain.
    Demodex mites are commensal organisms rarely found in healthy children. Human demodicosis can be classified as a primary or a secondary form. The secondary form in children usually affects severely immunodepressed children. Read More

    Langerhans Cell Histiocytosis With Clinical and Histologic Features of Hidradenitis Suppurativa: Brief Report and Review.
    Am J Dermatopathol 2017 Sep 11. Epub 2017 Sep 11.
    Department of Dermatology, University of Florida College of Medicine, Gainesville, FL.
    Langerhans cell histiocytosis (LCH) is an uncommon histiocytic disorder in adults. Clinically, this rare entity can mimic other dermatologic conditions, including hidradenitis suppurativa. A case of LCH is reported with clinical and histologic features of hidradenitis suppurativa, along with a review of these unusual findings. Read More

    Langerhans cell histiocytosis limited to the female genital tract: A review of literature with three additional cases.
    Gynecol Oncol Rep 2017 Nov 26;22:4-8. Epub 2017 Aug 26.
    Department of Pathology and Laboratory Medicine, The University of Vermont Medical Center, Burlington, VT, USA.
    •LCH of the female reproductive tract has four patterns of involvement.•A comprehensive literature review revealed 35 cases of pure genital LCH.•We report two new cases of pure LCH lesions of the vulva and one of the cervix. Read More

    Clinical course of the bony lesion of single-system single-site Langerhans cell histiocytosis - Is appropriate follow-up sufficient treatment?
    J Orthop Sci 2017 Sep 16. Epub 2017 Sep 16.
    Department of Orthopaedic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan.
    Background: Langerhans cell histiocytosis (LCH) is categorized into three types, which include single-system single-site (SS-s), single-system multiple-site (SS-m) and multisystem (MS). The most commonly affected site in LCH is bone, and the bony lesion of SS-s LCH has a good prognosis. The bony lesion of SS-s LCH has been thought to regress spontaneously. Read More

    Evaluation of pituitary uptake incidentally identified on (18)F-FDG PET/CT scan.
    Oncotarget 2017 Aug 16;8(33):55544-55549. Epub 2017 Feb 16.
    Department of Nuclear Medicine, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
    The clinical significance of pituitary uptake on routine whole body (18)F-fluorodeoxyglucose (FDG) positron emission tomography/computer tomography (PET/CT) is not completely characterized. We seek to assess the potential differential diagnosis/underlying etiology of pituitary FDG uptake incidentally identified on routine PET/CT scans. A total of 24,007 PET/CT whole body scans in recent 5 years were retrospectively reviewed. Read More

    Chronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor: Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia.
    J Cutan Pathol 2017 Sep 8. Epub 2017 Sep 8.
    Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas.
    Chronic myelomonocytic leukemia (CMML) is a hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features. Cutaneous involvement by CMML is critical to recognize as it typically is a harbinger of disease progression and an increased incidence of transformation to acute myeloid leukemia. Cutaneous lesions of CMML exhibit heterogeneous histopathologic features that can be challenging to recognize as CMML. Read More

    How I manage pulmonary Langerhans cell histiocytosis.
    Eur Respir Rev 2017 Sep 6;26(145). Epub 2017 Sep 6.
    National Reference Centre for Histiocytoses, Pulmonary Dept, Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Louis, Paris, France
    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare sporadic cystic lung disease of unknown aetiology that is characterised by the infiltration and destruction of the wall of distal bronchioles by CD1a(+) Langerhans-like cells. In adults, PLCH is frequently isolated and affects young smokers of both sexes. Recent multicentre studies have led to the more standardised management of patients in clinical practice. Read More

    Atlanto-axial langerhans cell histiocytosis in a child presented as torticollis.
    World J Clin Cases 2017 Aug;5(8):344-348
    Miniar Tfifha, Saoussen Abroug, Pediatrics Department, Sahloul University Hospital, Sousse 4054, Tunisia.
    Langerhans cell histiocytosis (LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma (EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many reports of patients with spine histiocytosis are well documented in the literature but it is not the case of atlantoaxial localization. Read More

    Erdheim-Chester Disease: The Importance of Information Integration.
    Case Rep Oncol 2017 May-Aug;10(2):613-619. Epub 2017 Jul 11.
    McGill University, Jewish General Hospital, Division of Rheumatology, Department of Medicine, Montreal, QC, Canada.
    Background: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. Read More

    Langerhans Cell Histiocytosis Mimicking Periapical Pathology in a 39-Year-Old Man.
    J Endod 2017 Aug 29. Epub 2017 Aug 29.
    Division of Oral and Maxillofacial Pathology, Columbia University College of Dental Medicine, New York, New York. Electronic address:
    Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type dendritic cells, with more than 50% of cases of LCH seen in children younger than 15 years of age. The most common clinical presentation of LCH is solitary or multiple bony lesions. The jaws are affected in approximately 10%-20% of cases, with a strong predilection for the mandible. Read More

    Refractory LCH With Secondary Intracranial PNET: A Case Report and Review of Literature.
    J Pediatr Hematol Oncol 2017 Aug 30. Epub 2017 Aug 30.
    *Apollo Center For Advanced Pediatrics †Department of Pediatric Hematology-Oncology, Indraprastha Apollo Hospital, New Delhi.
    The occurrence of second malignant neoplasms in patients with Langerhans cell histiocytosis is infrequent but has been reported. Here we report the case of a child with refractory Langerhans cell histiocytosis who was treated with cladribine and later developed a secondary intracranial primitive neuroectodermal tumor. The possible association of cladribine with second neoplasm is further discussed. Read More

    Langerhans cell histiocytosis of the thyroid complicated by papillary thyroid carcinoma: A case report and brief literature review.
    Medicine (Baltimore) 2017 Sep;96(35):e7954
    aDepartment of General Surgery bDepartment of Endocrinology cDepartment of Nuclear Medicine dDepartment of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
    Rationale: Langerhans cell histiocytosis (LCH) involves mainly the skin and bone and rarely the thyroid. Meanwhile, papillary thyroid carcinoma (PTC) is the most common subtype of thyroid cancer. Both LCH and PTC could make the thyroid enlarged and hypermetabolic. Read More

    CD207(+)CD1a(+) cells circulate in pediatric patients with active Langerhans cell histiocytosis.
    Blood 2017 Aug 28. Epub 2017 Aug 28.
    Hospital de Ninos Pedro de Elizalde, Buenos Aires, Argentina.
    Langerhans cell histiocytosis (LCH) is a rare disease with an unknown etiology characterized by heterogeneous lesions containing CD207(+)CD1a(+) cells that can arise in almost any tissue and cause significant morbidity and mortality. Precursors of pathological Langerhans cells have yet to be defined. Our aim was to identify circulating CD207(+)CD1a(+) cells and their inducers in LCH. Read More

    Systemic Juvenile Xanthogranuloma Involving the Bone Marrow, Multiple Bones, and the Skin That Developed During Treatment of Acute Lymphoblastic Leukemia in Remission State.
    Pediatr Dev Pathol 2017 Jan 1:1093526617721775. Epub 2017 Jan 1.
    1 Department of Pediatrics, 37977 School of Medicine, Ajou University , Suwon, Korea.
    Juvenile xanthogranuloma (JXG) is a rare benign disorder classified as non-Langerhans cell histiocytosis, with unclear etiology and pathogenesis. JXG is generally characterized by solitary or multiple cutaneous nodules that resolve spontaneously over a few years. JXG rarely presents as extracutaneous lesions that progress to a symptomatic systemic disorder through multiple organ involvement. Read More

    Intraosseous Rosai-Dorfman disease diagnosed by touch imprint cytology evaluation: A case series.
    Diagn Cytopathol 2017 Aug 23. Epub 2017 Aug 23.
    Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
    Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD) is a rare benign disorder that primarily affects the lymph nodes. Localized lymphadenopathy is the most common clinical manifestation of this disorder. However, RDD has been described in several extra-nodal sites including the head and neck region, soft tissue, skin, upper respiratory tract, gastro-intestinal tract and central nervous system (CNS). Read More

    Cutaneous Indeterminate Cell Histiocytosis of Donor Origin After Allogeneic Hematopoietic Stem-Cell Transplantation.
    Am J Dermatopathol 2017 Sep;39(9):696-699
    *Department of Pathology, Hospital Universitario de Salamanca, Salamanca, Spain;†Instituto de Investigación Biomédica de Salamanca (IBSAL), Salamanca, Spain;‡Dermatology Service, Hospital Universitario de Salamanca, Salamanca, Spain; and§Hematology Service, Hospital Universitario de Salamanca, Salamanca, Spain.
    Allogeneic hematopoietic stem-cell transplantation and solid-organ transplantation are associated with an increased risk of secondary neoplasms. Indeterminate cell histiocytosis (ICH) is a rare disease composed of so-called indeterminate cells, an alleged cutaneous dendritic cell subset displaying histological and some ultrastructural and immunophenotypic features of Langerhans cells but lacking Birbeck granules. We report a case of cutaneous ICH occurring after allogeneic hematopoietic stem-cell transplantation for a myelodysplastic syndrome in a 56-year-old man. Read More

    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 Sep 21;34(5):e288-e289. 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

    1 OF 211