scholarly journals “Langerhan Cell Histiocytosis –Review Of Literature”

2018 ◽  
Vol 1 (2) ◽  
Author(s):  
Ashutosh Jaysing Thorat ◽  
Pawan Vilasrao Dawane
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Manifestations ◽  
Disease Process ◽  
Surgical Excision ◽  
Langerhans Cell ◽  
Treatment Modalities ◽  
Review Of Literature ◽  
Immature Dendritic Cells ◽  
Wide Range ◽  
Criteria For Diagnosis

Background: Langerhans cell histiocytosis is a relatively rare unique disease process characterized by an abnormal proliferation of immature dendritic cells usually affecting children and young adults.Discussion:  Histiocytosis are rare diseases of great biological variability and a wide range of clinical manifestations. The first manifestations of LCH may occur in the oral cavity may vary from a continuous gingival infection or a dental abscess to necrotizing ulcerating defects or a painful jaw swelling.The criteria  for diagnosis of LCH includes identification of the characteristic clinical features histopathological, Immunohistochemical findings. Various treatment modalities has been adopted including wide surgical excision along with radiotherapy,chemotherapy ,isolated radiotherapy and use of alkalizing agents.Keywords- Langerhans cell histiocytosis, Histiocytosis X , Osteolysis of skull

scholarly journals Biological and clinical significance of somatic mutations in Langerhans cell histiocytosis and related histiocytic neoplastic disorders

Hematology ◽  
2015 ◽  
Vol 2015 (1) ◽  
pp. 559-564 ◽  
Author(s):  
Carl E. Allen ◽  
D. Williams Parsons
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Somatic Mutations ◽  
Mapk Pathway ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Common Mechanism ◽  
Recurrent Mutations ◽  
Wide Range ◽  
Neoplastic Disorders

AbstractLangerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG), and Erdheim–Chester disease (ECD) represent histiocytic disorders with a wide range of clinical manifestations. Until recently, mechanisms of pathogenesis have been speculative and debate has focused on classification of these conditions as reactive versus neoplastic. Genomic studies have been challenged by scarce tissue specimens, as well as heterogeneous nature of the lesions with variable infiltration of pathologic histiocytes. Whole-exome sequencing recently revealed a very low frequency of somatic mutations in LCH, JXG, and ECD compared to other neoplastic disorders. However, at least in the cases of LCH and ECD, there is a very high frequency of activating mutations in MAPK pathway genes, most notably BRAF-V600E, as well as MAP2K1, in LCH and NRAS in ECD. In ECD, recurrent mutations in the PI3K pathway gene PIK3CA have also been described. The heterogeneous clinical manifestations of these disorders may therefore be the cumulative result of activation of MAPK mutations (along with modifying signals from other pathways) at distinct stages of myeloid differentiation. Implications of this model include redefinition of LCH, JXG, and ECD as a group of clinically diverse myeloid neoplastic disorders with a common mechanism of pathogenesis. This model supports refocusing therapeutic strategies for these diseases on a personalized approach based on specific mutations and the cell(s) of origin.

scholarly journals Clinical characteristics and outcomes of Langerhans cell histiocytosis at a single institution in Thailand: a 20-year retrospective study

2021 ◽  
Vol 15 (4) ◽  
pp. 171-181
Author(s):  
Ponrachet Kitticharoenjit ◽  
Nucharin Supakul ◽  
Piya Rujkijyanont ◽  
Chanchai Traivaree ◽  
Apichat Photia ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Characteristics ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Organ Involvement ◽  
Bone Imaging ◽  
X Ray ◽  
Wide Range ◽  
Conjugated Hyperbilirubinemia

Abstract Background Langerhans cell histiocytosis (LCH) is a rare disease characterized by the various systems involved and clinical manifestations with a wide range of symptoms. Objectives To describe clinical characteristics, imaging, treatment, and outcomes of pediatric LCH at Phramongkutklao Hospital, Bangkok, Thailand. Methods We conducted a 20-year retrospective review of the medical records of patients diagnosed with LCH from birth to 21 years old from January 1, 1997, to December 31, 2016. Results In all, 14 patients with median age of 2.5 years were studied. Six (43%) patients had single-system (SS) LCH. Five patients (63%) with multisystem (MS) LCH (n = 8. 57%) had risk-organ involvement (RO+). All patients had plain X-ray imaging of their skull with 11 (79%) showing abnormal findings. Tc-99m bone imaging and fluorodeoxyglucose F18 (FDG) positron emission tomography (PET)-computed tomography (CT) demonstrated abnormal findings in 8 (89%) and 4 (29%) patients, respectively. The 5-year event-free survival (EFS) for patients with RO+ MS-LCH was less than that for those without risk-organ involvement (RO−) MS-LCH and SS-LCH (20% vs. 100%, P = 0.005). Hematological dysfunction, hypoalbuminemia, and conjugated hyperbilirubinemia may be worse prognostic factors for RO+ MS-LCH. Conclusion FDG-PET-CT might have a greater accuracy to detect LCH disease than conventional plain X-ray and Tc-99m bone imaging. RO+ MS-LCH has been encountered with relapse and poor outcomes. Hematopoietic involvement, hypoalbuminemia, and conjugated hyperbilirubinemia may be worse prognostic factors for RO+ MS-LCH.

EVOLUTION OF TREATMENT OF CHILDREN WITH LANGERHANS CELL HISTIOCYTOSIS

2021 ◽  
Vol 100 (3) ◽  
pp. 107-120
Author(s):  
M. Minkov ◽  
◽  
◽  
Keyword(s):  
Intracellular Signaling ◽  
Langerhans Cell Histiocytosis ◽  
Standard Treatment ◽  
Organs At Risk ◽  
Severe Disease ◽  
Clinical Manifestations ◽  
Maintenance Phase ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Wide Range

Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm caused by mutations in proteins in the intracellular signaling MAPK (mitogen-activated protein kinase) pathways. Its rarity, wide range of clinical manifestations, and varied course made it difficult to conduct clinical trials. The use of different stratification systems made it even more difficult to compare the published results. The review attempts to summarize approaches to treatment of LCH in children since the 1960s. Materials and methods of research: searches for original papers in PubMed were performed using combinations of the keywords Langerhans cell histiocytosis, X histiocytosis, pediatrics, children, treatment, and therapy. Only full articles published in English and German languages were included in the review. Conclusion: a combination of vinblastine and prednisolone as an intensive initial phase (6–12 weeks) followed by a less intensive maintenance phase of 12 months is the standard treatment of disseminated LCH. Mortality in multisystem LCH is caused by damage to the organs at risk (hematopoiesis, liver and spleen) and is especially high (up to 60–70%) if the patient does not respond to standard treatment. In the remaining patients, mortality is almost negligible, with the main problems being the recurrence of the disease and its irreversible effects in 50% of the survivors. Although systemic therapy has clearly improved survival in patients with the most severe disease, its role in sustaining disease control and prevention of irreversible consequences in patients with low-risk disease is less obvious.

Magnetic Resonance Imaging Features in Solitary Cerebral Langerhans Cell Histiocytosis: Case Report and Review of Literature

2018 ◽  
Vol 116 ◽  
pp. 333-336 ◽  
Author(s):  
Ju-Hwi Kim ◽  
Woo-Youl Jang ◽  
Tae-Young Jung ◽  
Kyung-Sub Moon ◽  
Shin Jung ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Case Report ◽  
Magnetic Resonance ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Imaging Features ◽  
Review Of Literature ◽  
Resonance Imaging

Laryngeal Langerhans Cell Histiocytosis: A Case Report and Literature Review

2020 ◽  
pp. 000348942095488
Author(s):  
Allen S. Zhou ◽  
Lei Li ◽  
Thomas L. Carroll
Keyword(s):  
Literature Review ◽  
Langerhans Cell Histiocytosis ◽  
Medical Center ◽  
Tertiary Care ◽  
Rare Condition ◽  
Surgical Excision ◽  
Langerhans Cell ◽  
Histopathological Analysis ◽  
Current Case ◽  
Tertiary Care Medical Center

Objective: To describe a case of laryngeal Langerhans cell histiocytosis, discuss its characteristic features and management, and provide a review of the available literature. Methods: A patient presenting to a tertiary care medical center with dyspnea and hoarseness is described. A literature review of laryngeal Langerhans cell histiocytosis cases was performed through a search of articles indexed in the National Institutes of Health PubMed system. Results: We report a case of a 69-year old male, who presented with a laryngeal mass highly suspicious for laryngeal squamous cell carcinoma, was treated with laser excision, and was subsequently found to have laryngeal Langerhans cell histiocytosis upon histological analysis. Including our current case, we found six prior reported cases of laryngeal Langerhans cell histiocytosis in the literature. Of the six cases, four were in adults, while two were in children. Dyspnea is a common presenting complaint present in all cases. Smoking may be a potential risk factor. Conclusions: Laryngeal Langerhans cell histiocytosis is a rare condition and an important consideration in the differential diagnosis of patients presenting with a laryngeal mass and symptoms of dyspnea or hoarseness. Biopsy and histopathological analysis are key to the diagnosis. Surgical excision and radiotherapy are successful treatments used in clinical practice.

scholarly journals A Case Report of Pediatric Langerhans Cell Histiocytosis: Current Approach and Diagnostic Challenges for Dermatologist

2020 ◽  
Vol 12 (3) ◽  
pp. 79-86
Author(s):  
Irwan Junawanto ◽  
Khairuddin Djawad ◽  
Sri Rimayani ◽  
Farida Tabri ◽  
Nurelly N. Waspodo ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Poor Prognosis ◽  
Clinical Manifestations ◽  
Current Approach ◽  
Myeloproliferative Disorder ◽  
Langerhans Cell ◽  
Histopathologic Examination ◽  
Chemotherapy Treatment ◽  
Cutaneous Manifestations ◽  
Patient Organ

Abstract Langerhans Cell Histiocytosis (LCH) is a chronic and rare myeloproliferative disorder caused by disorders in Lang-erhans cell proliferation in various organs and tissues. LCH has a wide variety of clinical manifestations, making it difficult to diagnose. Cutaneous manifestations are polymorphic in the form of purpura, papule, vesicles and pustules. LCH can involve vital organs such as the liver and lungs as well as the hematopoiesis system that usually gives a poor prognosis. The prognosis is also influenced by the age of patient, organ dysfunction and response to the first 6 weeks of chemotherapy treatment. A 3-year-old girl reported a major complaint of an abscess-like lesion in the region of neck accompanied by an extensive purpura of scalp, neck and inguinal areas accompanied by vulvar erosions. The immunohistochemical and histopathologic examination support LCH and the clinical improvement after intravenous administration of intravenous 3 mg/m2 Vinblastine chemotherapy, 75 mg/m2 etoposide, oral 40 mg/m2 per prednisone. After the 6th cycle of chemotherapy, the patient died.

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