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The New England Journal of Medicine Aug 2018
Review
Topics: Cell Differentiation; Histiocytosis, Langerhans-Cell; Humans; Mitogen-Activated Protein Kinase Kinases; Myeloid Cells
PubMed: 30157397
DOI: 10.1056/NEJMra1607548 -
Journal of the American Academy of... Jun 2018Langerhans cell histiocytosis (LCH) is an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase pathway. When... (Review)
Review
Langerhans cell histiocytosis (LCH) is an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase pathway. When disease involves the skin, LCH most commonly presents as a seborrheic dermatitis or eczematous eruption on the scalp and trunk. Evaluation for involvement of other organ systems is essential, because 9 of 10 patients presenting with cutaneous disease also have multisystem involvement. Clinical manifestations range from isolated disease with spontaneous resolution to life-threatening multisystem disease. Prognosis depends on involvement of risk organs (liver, spleen, and bone marrow) at diagnosis, particularly on presence of organ dysfunction, and response to initial therapy. Systemic treatment incorporating steroids and cytostatic drugs for at least one year has improved prognosis of multisystem LCH and represents the current standard of care.
Topics: Adolescent; Biopsy, Needle; Child; Child, Preschool; Disease Progression; Female; Histiocytosis, Langerhans-Cell; Humans; Immunohistochemistry; Incidence; Male; Multimorbidity; Prognosis; Rare Diseases; Risk Assessment; Severity of Illness Index
PubMed: 29754885
DOI: 10.1016/j.jaad.2017.05.059 -
Advances in Respiratory Medicine 2017Pulmonary Langerhans' cell histiocytosis (PLCH) is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow... (Review)
Review
Pulmonary Langerhans' cell histiocytosis (PLCH) is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow derived Langerhans' cells, which carry mutations of BRAF gene and/or NRAS, KRAS and MAP2K1 genes. It occurs predominantly in young smokers, without gender predominance. The disease is characterised by formation of eosinophilic granulomas with the presence of Langerhans' cells infiltrating and destroying distal airways. High-resolution computed tomography of the chest (HRCT) plays an outstanding role in PLCH diagnosis. The typical radiological picture of PLCH is the presence of small intralobular nodules, often forming 'tree in bud' lesions, cavitated nodules, thin- and thick-walled cystic lesions frequently confluent. Definite diagnosis requires the finding of characteristic lesions in histological examination and demonstration of antigen CD1a or CD207 presenting cells in immunohistochemistry. Smoking cessation is the most important recommendation for PLCH patients. There are no evidence based data regarding systemic steroid therapy. The treatment of progressive PLCH is based on cladribine or cytarabine as salvage therapy. The prognosis is good, and over 85% of patients survive 10 years.
Topics: Adult; Bronchi; Female; Histiocytosis, Langerhans-Cell; Humans; Lung; Male; Risk Factors; Smoking; Tomography, Spiral Computed
PubMed: 29083024
DOI: 10.5603/ARM.a2017.0046 -
Biomedica : Revista Del Instituto... Sep 2020Langerhans cell histiocytosis is a rare pathology with different clinical manifestations in the neonatal period ranging from isolated bone lesions to systemic... (Review)
Review
Langerhans cell histiocytosis is a rare pathology with different clinical manifestations in the neonatal period ranging from isolated bone lesions to systemic compromise. We report a case of Langerhans cell histiocytosis including a literature review focused on the clinical manifestations, diagnosis, and treatment. A one-month-old patient was brought to medical consultation with lymphadenopathy and skin lesions, which were initially managed as an infectious pathology. The disease continued its progression without improvement with the treatment until the patient died due to respiratory failure. The lymph node and skin biopsies revealed infiltration of atypical cells with positive immunohistochemistry for S100, CD1, and CD68 confirming Langerhans cell histiocytosis. This disorder represents a great challenge and, therefore, it is important to alert and sensitize medical teams about it for timely diagnosis and management.
Topics: Biopsy; Cytomegalovirus Infections; Diagnosis, Differential; Disease Progression; Fatal Outcome; Histiocytosis, Langerhans-Cell; Humans; Infant; Lymphadenopathy; Male; Skin; Skin Diseases; Skin Diseases, Viral
PubMed: 33030824
DOI: 10.7705/biomedica.5150 -
Survey of Ophthalmology 2008The clinical manifestations of Langerhans cell histiocytosis have been recognized for more than a century. For most of that time, physicians have viewed the disease from... (Review)
Review
The clinical manifestations of Langerhans cell histiocytosis have been recognized for more than a century. For most of that time, physicians have viewed the disease from different perspectives, interpreting portions of its clinical spectrum as if they were distinct and unrelated entities. More recently, Langerhans cell histiocytosis has been unified into a single concept, though the disease continues to defy traditional classification. By most accounts, Langerhans cell histiocytosis appears to be a morphologically benign proliferation of inflammatory cells that escapes regulatory control mechanisms. Studies from patients with all stages of the disease, however, document clonal proliferation of immune processing cells (i.e., Langerhans cells), suggesting a malignant disease process. The most common ophthalmic manifestation of Langerhans cell histiocytosis is a solitary lesion of orbital bone, which typically responds to minimally invasive therapy. The best management of solitary orbital Langerhans cell histiocytosis is debatable and has been complicated by its recent designation as a risk factor for central nervous system disease. This article summarizes recent developments in understanding the biology of Langerhans cell histiocytosis, reviews its ophthalmic manifestations, prognosis, and the controversy surrounding treatment of isolated orbital disease.
Topics: Eye Diseases; Histiocytosis, Langerhans-Cell; History, 19th Century; History, 20th Century; Humans
PubMed: 18572052
DOI: 10.1016/j.survophthal.2008.04.007 -
Archives of Disease in Childhood Nov 2009Langerhans cell histiocytosis is a rare disease. Depending on which organs are involved, the disease may prove rapidly fatal, develop a chronic reactivating but... (Review)
Review
Langerhans cell histiocytosis is a rare disease. Depending on which organs are involved, the disease may prove rapidly fatal, develop a chronic reactivating but therapy-responsive pattern or resolve spontaneously. Understanding of the pathology of the disease is progressing rapidly, and while clinical trials of standard chemotherapy agents continue, it is likely that novel targeted therapy will become feasible in the next decade. Permanent consequences of the disease are more commoner than generally realised.
Topics: Adolescent; Child; Child, Preschool; Histiocytosis, Langerhans-Cell; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Prognosis; Tomography, X-Ray Computed
PubMed: 19457881
DOI: 10.1136/adc.2007.125872 -
The New England Journal of Medicine Dec 2022
Topics: Humans; Histiocytosis, Langerhans-Cell; Tomography, X-Ray Computed
PubMed: 36573701
DOI: 10.1056/NEJMicm2203885 -
British Journal of Haematology Dec 2019
Topics: Histiocytosis, Langerhans-Cell; Humans; Molecular Targeted Therapy; Mutation; Neoplasms; Neurodegenerative Diseases
PubMed: 31309539
DOI: 10.1111/bjh.16099 -
The New England Journal of Medicine Dec 2021
Topics: Exanthema; Female; Histiocytosis, Langerhans-Cell; Humans; Infant, Newborn; Remission, Spontaneous; Skin
PubMed: 34879451
DOI: 10.1056/NEJMicm2112460 -
The New England Journal of Medicine Jun 2000
Review
Topics: Adrenal Cortex Hormones; Histiocytosis, Langerhans-Cell; Humans; Langerhans Cells; Prognosis; Smoking; Smoking Cessation
PubMed: 10877650
DOI: 10.1056/NEJM200006293422607