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    [Zygomatic localization of Langerhans cell histiocytosis - a case report].
    Wiad Lek 2016 ;69(6):829-831
    Katedra i Zakład Podstawowych Nauk Medycznych, Wydział Zdrowia Publicznego w Bytomiu, Śląski Uniwersytet Medyczny w Katowicach, Polska.
    Langerhans cell histocytosis is a rare disease. LCH is reported mainly in children aged less than 15 years, however it may appear at any age. In this work, the case of 17-month boy is reported. Read More

    Histiocytoses: emerging neoplasia behind inflammation.
    Lancet Oncol 2017 Feb;18(2):e113-e125
    Service de Médecine Interne 2, Centre National de Référence Maladies Auto Immunes Systémiques Rares, Institut E3M, Hôpital Pitié-Salpêtrière, Paris, France.
    Histiocytoses are disorders characterised by inflammation and the accumulation of cells derived from the monocyte and macrophage lineages, which results in tissue damage. Although they are often considered rare disorders with protean clinical manifestations, considerable advances in the understanding of their genetics have led to increased clinical recognition of these conditions, and fuelled further insights into their pathogenesis. In this Review, we describe insights into the cells of origin, molecular pathology, clinical features, and treatment strategies for some of the most common histiocytic disorders, including Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease. Read More

    Common Skin Conditions in Children: Neonatal Skin Lesions.
    FP Essent 2017 Feb;453:11-17
    University of North Carolina Chapel Hill School of Medicine Dermatology Residency Program, 410 Market St. Suite 400 CB#7715, Chapel Hill, NC 27516.
    Skin findings during the initial month of life are ubiquitous. One study estimated that more than 95% of newborns have cutaneous findings, which often are distressing to parents but frequently are benign and self-limited. Among them are milia, cutis marmorata, congenital dermal melanocytosis, and the benign neonatal pustular eruptions (eg, benign cephalic pustulosis, erythema toxicum neonatorum, transient neonatal pustular melanosis). Read More

    Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while improving treatment outcomes.
    JCI Insight 2017 Feb 9;2(3):e89473. Epub 2017 Feb 9.
    Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center.
    Many patients with histiocytic disorders such as Langerhans cell histiocytosis (LCH) or Erdheim-Chester disease (ECD) have treatment-refractory disease or suffer recurrences. Recent findings of gene mutations in histiocytoses have generated options for targeted therapies. We sought to determine the utility of prospective sequencing of select genes to further characterize mutations and identify targeted therapies for patients with histiocytoses. Read More

    Traumatic Rib Injury: Patterns, Imaging Pitfalls, Complications, and Treatment.
    Radiographics 2017 Feb 10:160100. Epub 2017 Feb 10.
    From the School of Medicine and Dentistry, University of Rochester Medical Center, 601 Elmwood Ave, Box 648, Rochester, NY 14642.
    The ribs are frequently affected by blunt or penetrating injury to the thorax. In the emergency department setting, it is vital for the interpreting radiologist to not only identify the presence of rib injuries but also alert the clinician about organ-specific injury, specific traumatic patterns, and acute rib trauma complications that require emergent attention. Rib injuries can be separated into specific morphologic fracture patterns that include stress, buckle, nondisplaced, displaced, segmental, and pathologic fractures. Read More

    Effective BRAF inhibitor vemurafenib therapy in a 2-year-old patient with sequentially diagnosed Langerhans cell histiocytosis and Erdheim-Chester disease.
    Onco Targets Ther 2017 24;10:521-526. Epub 2017 Jan 24.
    Second Department of Pediatrics.
    Erdheim-Chester disease (ECD) is a rare histiocytic disorder, characterized by the xanthomatous infiltration of tissues by CD68-positive and CD1a-/CD100-negative foamy histiocytes. In childhood, ECD is exceptionally rare, and only a dozen cases have been published so far. The cooccurence of Langerhans cell histiocytosis (LCH) and ECD is even rarer. Read More

    Cutaneous Involvement in Adult Multisystemic Langerhans Cell Histiocytosis Successfully Treated With Pulsed Dye Laser.
    Dermatol Surg 2017 Feb 3. Epub 2017 Feb 3.
    Department of Dermatology, University Hospital of Salamanca, Salamanca, Spain Department of Pathology, University Hospital of Salamanca, Salamanca, Spain Department of Dermatology, University Hospital of Salamanca, Salamanca, Spain.

    [Pulmonary Langerhans' cell histiocytosis (PLCH) revealed by pneumothorax: about a case].
    Pan Afr Med J 2016 27;25:32. Epub 2016 Sep 27.
    Service de Pneumologie, Hôpital Arrazi, CHU Mohamed VI, Laboratoire PCIM, UCAM, Marrakech, Maroc.
    Langerhans cell histiocytosis is a rare disease of unknown etiology characterized by the infiltration of Langerhans cells in one or more organs. It has a polymorphic clinical presentation. We report the case of Mr R. Read More

    An unusual thoracic localizations of Erdheim- Chester disease: A case report.
    Respir Med Case Rep 2017 16;20:116-119. Epub 2017 Jan 16.
    Department of Diseases of the Thorax, GB Morgagni Hospital, Forlì, Italy; Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark.
    Erdheim- Chester disease is a rare non- Langerhans cell histiocytosis that usually involves the bones, heart, central nervous system, retroperitoneum, eyes, kidneys, skin and adrenals. Lungs are affected in up to one-half cases; at CT scan various patterns are described: interstitial disease, consolidations, micronodules and microcysts, with or without pleural involvement. We presented a case of a 59 year-old man with unusual intrathoracic manifestation of Erdheim- Chester disease. Read More

    Isolated Sphenoid Sinus Lesions: Experience with a Few Rare Pathologies.
    J Neurosci Rural Pract 2017 Jan-Mar;8(1):107-113
    Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
    Introduction: The sphenoid sinus is often neglected because of its difficult access. The deep position of the sphenoid sinus hinders early diagnosis of pathologies in that location. Delayed diagnosis can cause serious complications due to proximity to many important structures. Read More

    Bone Langerhans cell histiocytosis with pulmonary involvement in an adult non-smoker: A case report and brief review of the literature.
    Mol Clin Oncol 2017 Jan 10;6(1):67-70. Epub 2016 Nov 10.
    Department of Oncology, Zhongda Hospital Affiliated to Southeast University, Nanjing, Jiangsu 210009, P.R. China.
    Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause, which encompasses a set of disorders of multiple organs with various clinical presentations that share the common characteristic of sizeable Langerhans cell infiltration. The clinical spectrum of LCH ranges from solitary bone lesions to involvement of other organs. lung involvement in LCH may be part of a multisystem disease, which almost exclusively occurs in adult smokers, while it is overlooked or misdiagnosed in the majority of non-smokers. Read More

    Multisystem Langerhans Cell Histiocytosis in an Adult.
    Am J Med Sci 2017 Jan 3;353(1):94. Epub 2016 Aug 3.
    Division of Hematology/Oncology, Taipei Veterans General Hospital, Taipei, Taiwan; Institute of Biopharmaceutical Science, National Yang-Ming University, Taipei, Taiwan; Chong Hin Loon Memorial Cancer and Biotherapy Research Center, National Yang-Ming University, Taipei, Taiwan.

    Successful Treatment of Refractory Langerhans Cell Histiocytosis of the Choroid Plexus in a Child With Pulse Dexamethasone and Lenalidomide.
    J Pediatr Hematol Oncol 2017 Mar;39(2):e74-e78
    Departments of *Pediatric Hematology, Oncology and BMT †Radiology, Apollo Specialty Hospital, Chennai, Tamil Nadu, India.
    Refractory/relapsed Langerhans cell histiocytosis (LCH) has a difficult course with a guarded prognosis. Salvage treatments available are both expensive and highly toxic. On the basis of the pathophysiology of LCH, we used a novel protocol including pulse dexamethasone and lenalidomide in a child with refractory LCH involving the choroid plexus, which resulted in durable remission with minimal toxicity. Read More

    [Solitary and congenital juvenile xanthogranuloma: case report].
    Arch Argent Pediatr 2017 02;115(1):e9-e12
    Servicio de Dermatología, Hospital Nacional de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
    Juvenile xanthogranuloma is a bening pathology and it represents the most common form of non-Langerhans cell histiocytosis. It is characterized by the presence of papules or firm nodules of a pinkish or yellow-brownish nature, which mainly compromise the skin and, exceptionally, other organs. It is a self-limited entity having a spontaneous regression during the first five years of life. Read More

    Spectrum of Xanthogranulomatous Processes in the Abdomen and Pelvis: A Pictorial Review of Infectious, Inflammatory, and Proliferative Responses.
    AJR Am J Roentgenol 2017 Jan 17:1-10. Epub 2017 Jan 17.
    3 Department of Diagnostic Radiology, The University of Texas M. D. Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030.
    Objective: Xanthogranulomatous (XG) processes are rare inflammatory conditions with the characteristic pathologic feature of lipid-laden macrophages or histiocyte cells. Imaging findings are nonspecific and can simulate aggressive neoplastic processes. XG processes can be caused by infection, inflammation, histolytic process, or an inherited lysosomal disorder. Read More

    [Child Langerhans cell histiocytosis].
    Presse Med 2017 Jan 10;46(1):85-95. Epub 2017 Jan 10.
    Hôpital Trousseau, centre de référence des histiocytoses, service d'hémato-oncologie pédiatrique, 26, avenue du Dr-Netter, 75012 Paris, France.
    Definition: Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Read More

    Langerhans cell histiocytosis associated with lymphoma: an incidental finding that is not associated with BRAF or MAP2K1 mutations.
    Mod Pathol 2017 Jan 13. Epub 2017 Jan 13.
    Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
    Langerhans cell histiocytosis is characterized by a localized or systemic proliferation of Langerhans cells. BRAF mutations have been reported in 40-70% of cases and MAP2K1 mutations have been found in BRAF-negative cases, supporting that Langerhans cell histiocytosis is a true neoplasm, at least in mutated cases. In a small subset of patients, Langerhans cell histiocytosis is detected incidentally in a biopsy involved by lymphoma. Read More

    Pediatric Erdheim-Chester disease with aggressive skin manifestations.
    Br J Dermatol 2017 Jan 12. Epub 2017 Jan 12.
    Department of Dermatology, Tianjin Medical University General Hospital, Tianjin, China.
    Erdheim-Chester disease (ECD), one type of systemic non-Langerhans cell histiocytosis, is uncommon and characterized by the accumulation of CD68+/CD1a- foamy histiocytes. It is extremely rare in children. The skin lesions of pediatric ECD was not systemically described before. Read More

    Radiographic Differentiation of Advanced Fibrocystic Lung Diseases.
    Ann Am Thorac Soc 2017 Jan 11. Epub 2017 Jan 11.
    National Hospital Organization Kinki-Chuo Medical Center, Osaka, Japan ;
    The concept of end-stage lung disease suggests a final common pathway for most diffuse parenchymal lung diseases. In accordance with this concept, end-stage disease is characterized radiographically and pathologically by the presence of extensive honeycombing. However, sequential computed tomographic (CT) scans obtained from patients with chronic diffuse lung disease evolve over time to show various advanced lung disease patterns other than honeycombing. Read More

    Physiologic Determinants of Exercise Capacity in Pulmonary Langerhans Cell Histiocytosis: A Multidimensional Analysis.
    PLoS One 2017 10;12(1):e0170035. Epub 2017 Jan 10.
    Service de Pneumologie et ImmunoAllergologie, Centre de Compétence des Maladies Pulmonaires Rares, CHU Lille, Univ. Lille, Lille, France.
    Background: Reduced exercise capacity severely impacts quality of life in pulmonary Langerhans cell histiocytosis. Ascertaining mechanisms that impair exercise capacity is necessary to identify targets for symptomatic treatments.

    Methods: Dyspnea, pulmonary function tests and cardiopulmonary exercise test were analysed in 62 study participants. Read More

    Langerhans cell histiocytosis of skull: a retrospective study of 18 cases.
    Ann Palliat Med 2016 Nov 28. Epub 2016 Nov 28.
    Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
    Background: The present study presents 18 cases of Chinese patients harboring a Langerhans cell histiocytosis (LCH) of the skull.

    Methods: Eighteen consecutive patients were diagnosed as LCH of the skull and confirmed pathologically between March 2002 and February 2014. In the present study, the patients of LCH without skull involvement were excluded. Read More

    A Rare Cause of Proptosis in Childhood: Langerhans Cell Histiocytosis.
    Turk J Ophthalmol 2016 Aug 15;46(4):194-196. Epub 2016 Aug 15.
    Mersin University Faculty of Medicine, Department of Pathology, Mersin, Turkey.
    A three-year-old male patient was admitted to the clinic with proptosis in his right eye. He had a history of fever with an unknown etiology. In examination, right proptosis was observed and an immobile mass was palpated at the lateral wall of the right orbita. Read More

    Eruptive xanthogranuloma in a healthy adult male.
    J Cutan Pathol 2016 Dec 21. Epub 2016 Dec 21.
    Department of Dermatology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
    Xanthogranuloma is a benign, non-Langerhans cell histiocytosis primarily diagnosed in infants and children, although a subset occurs in adults. Multifocal eruptive presentation of xanthogranuloma is very rare with only 4 previous cases reported in the literature to our knowledge. We describe a case of eruptive xanthogranuloma in a 49-year-old man who presented with sudden onset of numerous asymptomatic, red-yellow to orange papules on the face, scalp, axilla, flank and scrotum. Read More

    Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report.
    Indian Dermatol Online J 2016 Nov-Dec;7(6):516-519
    Department of Skin and V.D., B. J. Govt. Medical College and Sassoon General Hospital, Pune, India.
    Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis. It is characterized by progressive appearance of papules and nodules without spontaneous resolution. We report a 60-year-old patient with novel clinical features in the form of extensive noduloulcerative lesions, ichthyotic patches, and laryngeal involvement culminating in fatal outcome prior to therapeutic intervention. Read More

    Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma: Current Understanding and Differential Diagnosis.
    J Clin Exp Hematop 2016 ;56(2):109-118
    Department of Laboratory Medicine, Japan Baptist Hospital.
    Langerhans cell (LC) histiocytosis (LCH) and LC sarcoma (LCS) are proliferative processes consisting of cells having morphologic and phenotypic features of Langerhans cells (LCs), although the latter may have lost some of these features. Because neoplastic nature of LCH as well as LCS is more likely by recent studies, a category of LC hyperplasia can be better characterized. LCH and LCS are rarely seen in daily pathology practice, but it is important to accurately characterize these lesions. Read More

    Predictor Variables of Developing Anterior Pituitary Deficiencies in a Group of Paediatric Patients with Central Diabetes Insipidus and Langerhans Cell Histiocytosis.
    Horm Res Paediatr 2016 Dec 13. Epub 2016 Dec 13.
    Endocrine Service, Hospital de Pediatria Garrahan, Buenos Aires, Argentina.
    Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unknown etiopathogenesis. Central diabetes insipidus (CDI) is the most frequent endocrine manifestation and is a known risk factor for the development of further anterior pituitary hormone deficiencies (APD). However, not all CDI patients develop APD, as observed during prolonged periods of follow-up. Read More

    Recurrence Pattern of Pulmonary Langerhans Cell Histiocytosis After Lung Transplantation: A Case Report.
    Transplant Proc 2016 Nov;48(9):3231-3233
    Turkiye Yuksek Ihtisas Training and Research Hospital, Thoracic Surgery and Lung Transplantation Clinic, Ankara, Turkey.
    Pulmonary Langerhans cell histiocytosis is an uncommon indication for lung transplantation. The recurrence of the primary pathology after transplantation is rare. Extra-pulmonary involvement of Langerhans cell histiocytosis before transplantation and resuming of smoking after transplantation are risk factors for recurrence. Read More

    Late-Onset Langerhans Cell Histiocytosis with Cerebellar Ataxia as an Initial Symptom.
    Case Rep Neurol 2016 Sep-Dec;8(3):218-223. Epub 2016 Oct 31.
    Department of Neurology, Seoul National University Hospital, College of Medicine, Seoul, South Korea.
    Late-onset progressive cerebellar ataxia is a diagnostic challenge because of a poor correlation between genotype and phenotype, and a broad range of secondary causes that extend beyond the neurological field. We report the case of a 45-year-old woman admitted after 2 years of slowly progressing cerebellar ataxia, dysarthria, and emotional instability. Notably, she was diagnosed with diabetes insipidus at the age of 35. Read More

    A rare case of extensive cranial Langerhans cell histiocytosis, synchronously presenting as otitis externa and giant cell arteritis.
    J Surg Case Rep 2016 Dec 1;2016(11). Epub 2016 Dec 1.
    Department of Otolaryngology, Worcestershire Acute Hospitals NHS Trust, Worcester, United Kingdom.
    Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unpredictable clinical course and varied modes of presentation. The spectrum of presentation is wide, ranging from isolated eosinophilic granulomas to multiple lesions and diffuse systemic involvement. We present the case of a 52-year-old man, who presented with an 8-week history of worsening otalgia and superficial temporal tenderness attributed to otitis externa within the community and subsequently giant cell arteritis. Read More

    Histopathological and Clinical Findings in Cutaneous Manifestation of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome Associated With the BRAFV600E Mutation.
    Am J Dermatopathol 2016 Nov 23. Epub 2016 Nov 23.
    *Dermatopathology Duisburg, Duisburg, Germany; and †Divisions of Dermatopathology and Dermatology, Department of Pathology, Albany Medical College, Albany, NY.
    The overlap of Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) is more common than it was generally accepted. Both diseases seem to be linked by a mutation in oncogenic BRAFV600E, probably an early event which occurs in bone marrow progenitor cells. In this article are described the clinical and histological findings in 2 cases of ECD-LCH overlap syndrome bearing the BRAFV600E mutation in both ECD and LCH lesions in bone and skin. Read More

    Usual and Unusual Manifestations of Familial Hemophagocytic Lymphohistiocytosis and Langerhans Cell Histiocytosis.
    Pediatr Clin North Am 2017 Feb;64(1):91-109
    Division of Pediatric Hematology/Oncology/Blood and Marrow Transplant, Medical College of Wisconsin, 8701 Watertown Plank Road, MFRC 3018, Milwaukee, WI 53226, USA. Electronic address:
    Familial hemophagocytic lymphohistiocytosis (FHL) and Langerhans cell histiocytosis (LCH) are histiocytic diseases that occur most commonly in young children. Improvements in recognition and treatment have been substantial for both diseases in the past decade, although early and late morbidity continue to be major concerns. These two diagnoses behave differently, although the clinical spectra for both diseases are diverse and can lead to confusion and delays in diagnosis and treatment. Read More

    "Histiocytosis X" - A Rare Case Report.
    J Clin Diagn Res 2016 Oct 1;10(10):ZD19-ZD22. Epub 2016 Oct 1.
    Postgraduate Student, Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Sri Ramachandra University , Chennai, Tamil Nadu, India .
    Histiocytosis X is an idiopathic disease, characterized by a disorder of the reticulo-endothelial system in the human body. Histopathological studies carried out right from the 1800s have seen a significant similarity in the pathologic process of different stages in particular clinical syndromes showing proliferation of mature histiocytes. It was then modified by Lichenstein in 1953 as "Histiocytosis X". Read More

    [Langerhans cell histiocytosis and Erdheim-Chester disease, a continuity?]
    Rev Med Interne 2016 Nov 23. Epub 2016 Nov 23.
    Service d'endocrinologie, hôpital du Cluzeau, 23, avenue Dominique-Larrey, 87042 Limoges cedex, France.
    Introduction: Erdheim-Chester disease and langerhans cell histiocytosis are two rare diseases separate on clinical, radiological and histological aspects. However, several cases involving both entities have been described.

    Observation: A 70-year-old man had a central diabetes insipidus, xanthelasmas, retroperitoneal fibrosis and osteosclerosis of the legs suggestive of Erdheim-Chester disease. Read More

    Multicentric reticulohistiocytosis: Rheumatology perspective.
    Best Pract Res Clin Rheumatol 2016 Apr 1;30(2):250-260. Epub 2016 Oct 1.
    Department of Internal Medicine, Division of Rheumatology, Istanbul Faculty of Medicine, Istanbul, Turkey. Electronic address:
    Multicentric reticulohistiocytosis (MRH) is a rare, multisystemic non-Langerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. There are no specific laboratory findings for MRH. Diagnosis is based on clinical findings and skin or synovial biopsy results. Read More

    Thyroid Langerhans cell histiocytosis and papillary thyroid carcinoma.
    Gland Surg 2016 Oct;5(5):537-540
    Histopathology Department, National Gaurd Hospital, King AbullAziz Medical City, Jeddah, Saudi Arabia.
    A 27-year-old female, married with two children, presented to our clinic with a 1-year history of thyroid swelling and pressure symptoms on lying backward and bilateral cervical lymphadenopathy. The patient was a known case of panhypopituitarism for 5 years. Comprehensive patient evaluation including FNAC with papillary thyroid cancer result then she underwent total thyroidectomy and bilateral neck dissection and final histologic examination confirmed papillary thyroid carcinoma in the background of lymphocytic thyroiditis, associated with Langerhans cell histiocytosis (LCH). Read More

    Retrospective analysis of nonendodontic periapical lesions misdiagnosed as endodontic apical periodontitis lesions in a population of Taiwanese patients.
    Clin Oral Investig 2016 Nov 16. Epub 2016 Nov 16.
    School of Dentistry, College of Dental Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
    Objective: We aimed to evaluate nonendodontic periapical lesions clinically misdiagnosed as endodontic periapical pathoses in a population of Taiwanese patients.

    Materials And Methods: Cases (2000-2014) of histopathological diagnoses of nonendodontic periapical lesions were retrieved from all cases with a clinical diagnosis of radicular cyst, apical granuloma, or apical periodontitis in the institution. These cases were regarded as misdiagnosed nonendodontic periapical lesions, of which the types and frequencies, in addition to the demographic data, were determined. Read More

    The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) - Clinical Aspects and Therapeutic Approaches.
    Klin Padiatr 2016 Nov 15;228(6-07):294-306. Epub 2016 Nov 15.
    Division of Pediatric Hematology and Oncology, Hospital for Children and Adolescents, Johann Wolfgang Goethe-University, Frankfurt, Germany.
    Rare histiocytoses, also called non-Langerhans cell histiocytoses, include all proliferative disorders of histiocytes, macrophages and dendritic cells that are not classified as Langerhans cell histiocytosis (LCH) and do not belong to the hemophagocytic lymphohistiocytosis (HLH) group of diseases. Thus, the term includes numerous benign or malignant, localized or systemic, adult or pediatric diseases. The classification of the histiocytic disorders has been revised several times. Read More

    Central diabetes insipidus: clinical profile that suggests organicity in Peruvian children: Lima - Peru 2001-2013.
    J Pediatr Endocrinol Metab 2016 Dec;29(12):1353-1358
    Background: Central diabetes insipidus (CDI) is a heterogeneous disease caused by arginine vasopressin deficiency; its management implies a profound understanding of the pathophysiology and the clinical spectrum. The aim of the study was to describe the clinical characteristics that indicate organicity in children and adolescents with central diabetes insipidus treated at the Department of Endocrinology from The Child Health's Institute during 2001 to 2013.

    Methods: Cross-sectional, retrospective study. Read More

    Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease.
    Case Rep Neurol Med 2016 20;2016:1030478. Epub 2016 Oct 20.
    Dipartimento di Biomedicina Sperimentale e Neuroscienze Cliniche, Università degli Studi di Palermo, Palermo, Italy.
    Background. Rosai-Dorfmann disease (RDD) is a rare, idiopathic non-Langerhans cell histiocytosis, affecting children and young adults, that commonly presents as painless, massive cervical lymphadenopathy with fever, weight loss, and polyclonal hypergammaglobulinemia. Cervical lymphadenopathy and extranodal involvement are the main presentations. Read More

    A Fatal Case of Congenital Langerhans Cell Histiocytosis with Disseminated Cutaneous Lesions in a Premature Neonate.
    Case Rep Pediatr 2016 19;2016:4972180. Epub 2016 Oct 19.
    Department of Laboratory Medicine, Uji-Tokushukai Medical Center, Uji, Kyoto 611-0042, Japan.
    Background. The outcome of neonates with congenital cutaneous Langerhans cell histiocytosis (LCH) is variable. Observations. Read More

    High frequency of clonal IG and T-cell receptor gene rearrangements in histiocytic and dendritic cell neoplasms.
    Oncotarget 2016 Nov;7(48):78355-78362
    Department of Pathology, National Cancer Center, Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
    The 2008 World Health Organization (WHO) diagnostic criteria of histiocytic and dendritic cell neoplasms from hematopoietic and lymphoid tissues no longer required the absence of clonal B-cell/T-cell receptor gene rearrangements. It is true that the clonal B-cell/T-cell receptor gene rearrangements have been identified in rare cases of histiocytic and dendritic cell neoplasms, such as those with or following lymphoma/leukemia or in some sporadic histiocytic/dendritic cell sarcomas, but the clonal features of such group of tumor are still not clear. Here we investigated the clonal status of 33 samples including Langerhans cell histiocytosis (LCH), Langerhans cell sarcoma (LCS), follicular dendritic cell sarcoma (FDCS), interdigitating dendritic cell sarcoma (IDCS) and histiocytic sarcoma (HS). Read More

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