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Indian Journal of Cancer 2022Langerhans cell histiocytosis (LCH) is a rare monoclonal disease of antigen presenting cells. Involvement of thyroid gland by LCH is exceedingly rare. Herein, we present... (Review)
Review
Langerhans cell histiocytosis (LCH) is a rare monoclonal disease of antigen presenting cells. Involvement of thyroid gland by LCH is exceedingly rare. Herein, we present a case of LCH involving the thyroid in a 38-year-old woman. Our patient presented clinically as a case of primary thyroid neoplasm. Presence of elongated, epithelioid neoplastic cells with grooved nuclei along with presence of background eosinophils were seen on fine-needle aspiration cytology (FNAC) and histopathology. Positive staining for CD1a and S100 immunohistochemistry confirmed the diagnosis of LCH. Patient was given combination chemotherapy and has responded well to the same without any complaints for last 6 months. With this report, our goal is to expand awareness of this rare tumor in the thyroid. Consideration at the time of FNAC and its correct diagnosis on subsequent excision is imperative for patient management.
Topics: Adult; Biopsy, Fine-Needle; Cytodiagnosis; Female; Histiocytosis, Langerhans-Cell; Humans; Thyroid Neoplasms
PubMed: 35645052
DOI: 10.4103/ijc.IJC_608_20 -
The Veterinary Clinics of North... Jan 2023Canine cutaneous histiocytomas originate from Langerhans cells. Multiple histiocytomas are referred to as cutaneous Langerhans cell histiocytosis. Feline pulmonary... (Review)
Review
Canine cutaneous histiocytomas originate from Langerhans cells. Multiple histiocytomas are referred to as cutaneous Langerhans cell histiocytosis. Feline pulmonary Langerhans cell histiocytosis causes respiratory failure owing to extensive lung infiltration. Localized and disseminated histiocytic sarcomas usually arise from interstitial dendritic cells. Primary sites include spleen, lung, skin, brain (meninges), lymph node, bone marrow, and synovial tissues of limbs. An initially indolent form of localized histiocytic sarcomas, progressive histiocytosis, originates in the skin of cats. Hemophagocytic histiocytic sarcomas originates in splenic red pulp macrophages. Canine reactive histiocytoses (systemic histiocytosis and cutaneous histiocytosis) are complex inflammatory diseases with underlying immune dysregulation.
Topics: Dogs; Cats; Animals; Histiocytic Sarcoma; Dog Diseases; Histiocytosis, Langerhans-Cell; Skin; Skin Neoplasms; Cat Diseases
PubMed: 36270835
DOI: 10.1016/j.cvsm.2022.07.010 -
Clinical Advances in Hematology &... Feb 2019Langerhans cell histiocytosis (LCH) is an inflammatory neoplasm of myeloid origin characterized by the presence of classic CD1a+/CD207+ cells. An ongoing debate over the... (Review)
Review
Langerhans cell histiocytosis (LCH) is an inflammatory neoplasm of myeloid origin characterized by the presence of classic CD1a+/CD207+ cells. An ongoing debate over the grouping of LCH was finally settled in favor of neoplasm after the discovery of the BRAF V600E mutation in 2010. The pathologic cells were found to involve an almost universal activation of the MAPK/ERK pathway, with mutations identified in most kinases upstream of ERK (RAS/RAF/MEK). The clinical presentation of LCH is a mixed bag, ranging from self-resolving localized disease to fulminant, fatal disseminated disease. The current standard of care for patients with multisystem LCH, who have high relapse rates, continues to be combination treatment with vinblastine and prednisone. Patients treated with BRAF and MEK inhibitors have shown a significant and sustained response in early-phase trials. During the current decade, researchers have described an extensive genomic landscape for LCH that has significantly enlarged our understanding of the biology and pathogenesis of this disease, especially neurodegenerative LCH. These advances have opened the door to studies of precision medicine and targeted therapy in LCH. Disease reactivation, long-term sequelae, very high-risk disease, and neurodegenerative LCH represent ongoing challenges. A renewed understanding of the biology of this disease, coupled with targeted therapies, may help in overcoming most of these challenges.
Topics: Biomarkers; Child; Disease Management; Disease Susceptibility; Histiocytosis, Langerhans-Cell; Humans; MAP Kinase Signaling System; Molecular Targeted Therapy; Mutation; Phenotype; Severity of Illness Index
PubMed: 30845115
DOI: No ID Found -
Archives de Pediatrie : Organe Officiel... Jan 1996Histiocytosis X, or Langerhans cell histiocytosis (LCH), is a rare disease, with an estimated incidence of 1/200,000 per year in children under 15 years of age. It has a... (Review)
Review
Histiocytosis X, or Langerhans cell histiocytosis (LCH), is a rare disease, with an estimated incidence of 1/200,000 per year in children under 15 years of age. It has a wide clinical spectrum, from single bone involvement (eosinophilic granuloma) to multisystemic disease with organ failure. The treatment of LCH is still controversial. While single system disease may spontaneously recover or requires minimal treatment, some multisystemic disease with organ dysfunction may resist systemic chemotherapy. The mortality rate in this latter subgroup of patients is relatively high and reaches 40% of the cases. A rational approach to the treatment of LCH is based on a better understanding of clinical and pathophysiological features. A prospective multicentre trial including scientific research should be planned, in order to determine the optimal treatment of LCH and to ameliorate the prognosis of the severe unresponsive form of the disease.
Topics: Child, Preschool; Histiocytosis, Langerhans-Cell; Humans; Infant; Prognosis
PubMed: 8745830
DOI: 10.1016/s0929-693x(96)80012-1 -
Clinics in Chest Medicine Sep 2004Pulmonary Langerhans' cell histiocytosis (PLCH) is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers.... (Review)
Review
Pulmonary Langerhans' cell histiocytosis (PLCH) is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers. PLCH belongs to the spectrum of Langerhans' cell histiocytosis (LCH), diseases characterized by uncontrolled proliferation and infiltration of various organs by Langerhans' cells. Other clinical entities within this spectrum of LCH are seen in adults and children and vary in severity from mild disease that requires no therapy to severe disseminated forms with extensive organ involvement and high mortality. Organ systems involved by LCH may include skin, bone, pituitary gland, lymph nodes, and lungs. Although LCH is approximately three times more common in children than adults, pulmonary involvement is much more common in adults with LCH, in whom it frequently occurs as the sole organ involved with disease. This article summarizes recent advances and current understanding of PLCH.
Topics: Algorithms; Bronchoscopy; Dendritic Cells; Disease Progression; Histiocytosis, Langerhans-Cell; Humans; Smoking; Tomography, X-Ray Computed
PubMed: 15331192
DOI: 10.1016/j.ccm.2004.04.005 -
Anales de Pediatria Aug 2022Langerhans cell histiocytosis (LCH) is a type of myeloid neoplasia that can affect different organs or tissues and exhibits substantial variability in its clinical...
Langerhans cell histiocytosis (LCH) is a type of myeloid neoplasia that can affect different organs or tissues and exhibits substantial variability in its clinical presentation and biological behaviour, so it may mimic different diseases. Performance of different clinical assessments and laboratory and imaging tests is recommended to determine the extent of involvement, which may be of a single location or multisystemic, and the presence or absence of dysfunction in risk organs, such as the haematopoietic system, liver and spleen. The diagnosis must be confirmed by histological examination of a biopsy sample. Molecular tests have identified mutations in the mitogen-activated protein kinase (MAPK) pathway, which has expanded treatment options. The diagnosis is complex and there is controversy regarding the management of certain cases. Treatment recommendations depend on the location of the lesions and the extent of involvement. International collaborative studies have demonstrated the effectiveness of prolonged combination therapies such as vinblastine and prednisone in severe or multisystemic forms, and anti-inflammatory drugs such as indomethacin and other cytostatic combinations have proven beneficial. Langerhans cell histiocytosis is a good example of the importance of precision medicine and the benefit of identifying molecular targets, common to different neoplasms, to develop new therapies. MAPK pathway inhibitors offer an alternative treatment option in refractory cases and neurodegenerative forms of LCH. Molecular testing can contribute to the prognosis, treatment and follow-up of LCH, especially in severe forms of disease.
Topics: Combined Modality Therapy; Histiocytosis, Langerhans-Cell; Humans; Mutation; Neoplasms; Prognosis
PubMed: 35869015
DOI: 10.1016/j.anpede.2022.05.005 -
Journal Francais D'ophtalmologie Sep 2022
Topics: Histiocytosis, Langerhans-Cell; Humans; Orbital Diseases
PubMed: 35786521
DOI: 10.1016/j.jfo.2022.01.011 -
Revue Des Maladies Respiratoires Mar 2023
Review
Topics: Humans; Histiocytosis, Langerhans-Cell; Lung; Lung Transplantation
PubMed: 36868974
DOI: 10.1016/j.rmr.2023.01.022 -
Fetal and Pediatric Pathology Jun 2023Langerhans cell histiocytosis (LCH) has heterogeneous presentations involving single or multiple systems, but simultaneous isolated skin and gastrointestinal... (Review)
Review
Langerhans cell histiocytosis (LCH) has heterogeneous presentations involving single or multiple systems, but simultaneous isolated skin and gastrointestinal involvement is not common. A female infant with intermittent bloody diarrhea was unresponsive for treatment of food allergy. Histology of gastric and colonic tissues demonstrated Langerhan's cell histiocytosis. The infant also had red rashes that were histologically proven Langerhan's cell histiocytosis. Chemotherapy utilized vincristine, cytarabine and prednisone. The bloody diarrhea and rash completely resolved with no recurrence in the 11 months of follow-up. Langerhan cell histiocytosis may present with simultaneous gastrointestinal and skin involvement.
Topics: Infant; Humans; Child; Female; Skin; Histiocytosis, Langerhans-Cell; Diarrhea; Colon
PubMed: 36331524
DOI: 10.1080/15513815.2022.2142488 -
La Revue de Medecine Interne Oct 2015Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells, most often... (Review)
Review
Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells, most often organized in granulomas. The disease has been initially described in children. The clinical picture of LCH is highly variable. Bone, skin, pituitary gland, lung, central nervous system, lymphoid organs are the main organs involved whereas liver and intestinal tract localizations are less frequently encountered. LCH course ranges from a fulminant multisystem disease to spontaneous resolution. Several randomized controlled trials have enable pediatricians to refine the management of children with LCH. Adult LCH has some specific features and poses distinct therapeutic challenges, knowing that data on these patients are limited. Herein, we will provide an overview of current knowledge regarding adult LCH and its management. We will also discuss recent advances in the understanding of the disease, (i.e. the role of BRAF oncogene) that opens the way toward targeted therapies.
Topics: Adult; Age of Onset; Diagnosis, Differential; Histiocytosis, Langerhans-Cell; Humans; Neoplasms
PubMed: 26150351
DOI: 10.1016/j.revmed.2015.04.015