scholarly journals Clinical characteristics and survival of children with Langerhans cell hystiocytosis

2008 ◽  
Vol 136 (9-10) ◽  
pp. 514-518
Author(s):  
Nada Krstovski ◽  
Dragana Janic ◽  
Lidija Dokmanovic ◽  
Radivoj Brdar
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Characteristics ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Langerhans Cell ◽  
Single System ◽  
Multisystem Disease ◽  
System Disease ◽  
The Central Nervous System

INTRODUCTION Langerhans cell histiocytosis is a rare disease in children, initial presentation is variable, clinical course, prognosis and survival are mostly unpredictable. OBJECTIVE To summarise clinical characteristics and treatment results in children with Langerhans cell histiocytosis. METHOD Retrospectively there were analyzed patients with LCH diagnosed and treated at Hematology Department of University Children's Hospital in Belgrade from 1990 to 2006. Clinical presentation, therapy and survival according to Kaplan-Meier's statistical test was analysed. RESULTS 30 patients were treated, aged from 4 months to 14 years, mean 3.9 years, median 2.3 years, 18 (60%) males, 12 (40%) females. A single system disease was diagnosed in 16 (53%) patients, of whom 6 patients with multifocal bone disease. All patients were in complete remission averagely following162 and 82 months respectively. Multisystem disease was found in 14 (47%) patients. The lymph nodes and skin were more frequently involved organs than the central nervous system (diabetes insipidus), lung, liver and spleen. The number of involved organs ranged from 2 to 8, mean 4.2. Four patients died due to disease progression 3, 16, 36 and 66 months after diagnosis. Nine patents with multisystem disease were in remission with 117 months of follow-up. One patient was lost on follow-up. CONCLUSION The clinical course of patients with a single system disease is usually benign while a multisystem disease has to be aggressively treated with precise initial evaluation and staging before therapy.

scholarly journals Langerhans Cell Histiocytosis - A Case Report

1970 ◽  
Vol 27 (2) ◽  
pp. 87-89
Author(s):  
SM Gurubacharya ◽  
RL Gurubacharya
Keyword(s):  
Organ Dysfunction ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Course ◽  
Eosinophilic Granuloma ◽  
Langerhans Cell ◽  
Class I ◽  
Unknown Etiology ◽  
Single System ◽  
Multisystem Disease ◽  
System Disease

Histiocytosis is a heterogenous group of disorders that are characterized by proliferation and activation of mononuclear phagocyte system. Langerhans Cell Histiocytosis (LCH) or Class I histiocytosis is a rare disorder of unknown etiology with proliferation of Langerhan cells which may infiltrate a single or multiple organs. This disease is more common in infants and children. It is usually sporadic but a familial pattern is known. The term embraces the whole clinical spectrum of the disorder from single bone lesions (eosinophilic granuloma) to an aggressive widespread multisystem disease in very sick child (Letterer-Siwe disease) with a wide variety of intermediate forms including the Hand-shuller Christian triad. The cause of LCH is not firmly established and most investigators in the field have long suspected that LCH is immunologic disorder either in its etiology or in its pathophysiology. Recent evidence suggests that LCH is a clonal disorder rather than reactive disease. LCH is classified according to sites of involvement into single system disease and multisystem disease. Single system disease can be either unifocal or multifocal. Multisystem disease can be either without organ dysfunction or with organ dysfunction. Clinical course of LCH with single system disease is usually benign with high chance of survival. However, its clinical course is often unpredictable and patients can experience spontaneous remission and exacerbations. Histiocytic diseases are currently classified by the writing group of the Histiocyte Society in the three classes, namely;Class I: Langerhans Cell Histiocytosis (LCH) Class II: Histiocytosis of mononuclear phagocytes other than Langerhans Cells Class III: Malignant Histiocytic disorders Key words: Langerhans Cell Histiocytosis, Eosinophilic granuloma, Skull, lytic lesion doi:10.3126/jnps.v27i2.1587 J. Nepal Paediatr. Soc. Vol.27(2) p.87-89

scholarly journals CLINICAL FEATURES AND OUTCOME IN CHILDREN WITH LANGERHANS CELL HISTIOCYTOSIS. A SINGLE INSTITUTION EXPERIENCE FROM PAKISTAN

2022 ◽  
Vol 71 (6) ◽  
pp. 2194-2201
Author(s):  
Nida Noor ◽  
Rabia Muhammad Wali ◽  
Annis -Ur- Rehman ◽  
Muhammad Abu Bakar
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Single System ◽  
Single Institution ◽  
Multisystem Disease ◽  
Disease Mortality ◽  
Multisystem Involvement ◽  
Related Mortality

Objective: To find out the clinical manifestations, treatment given and outcome of children with diagnosis of Langerhans cell histiocytosis. Study Design: Retrospective observational study. Place and Duration of Study: Shaukat Khanum Cancer Hospital, Lahore Pakistan, from Jan 2005 to Dec 2015. Methodology: Medical charts were reviewed in detail along with the available imaging for the patients. The data included age at the time of diagnosis, extent of the disease, involvement of risk organs, treatment given, response at 6th week of chemotherapy and at the end of the treatment, and outcome in terms of disease progression during the treatment, relapse of disease on follow up and cause of death either due to treatment related mortality or disease complications. Results: There were 29 patients, 12 patients (41%) had single system and 17 (58%) had multisystem involvement. 7 patients (41%) had risk organ involvement in the multisystem group. All the patients of multisystem and 6 patients of single system were treated according to the Langerhans cell histiocytosis III protocol. Commonest sites of involvement were bone in 22 (75%), followed by lymph nodes in 18 (62%) patients. Disease relapse was seen in 6 patients and all of them had multisystem disease. Mortality was observed only in multisystem Langerhans cell histiocytosis patients and more than 50% were risk organ positive. Conclusion: Langerhans cell histiocytosis is a highly heterogeneous disease. Some forms are curable without chemotherapy, while the multisystem disease requires aggressive treatment. However, despite intensive treatment, the multisystem disease and risk organs involved have poor...........

scholarly journals Retrospective analysis of clinical manifestations and treatment outcomes of patients diagnosed with langerhans cell histiocytosis from a tertiary cancer hospital in South India

2020 ◽  
Vol 8 (6) ◽  
pp. 2128
Author(s):  
Roshan Koshy Jacob ◽  
Shashidhar V. Karpurmath ◽  
Manjunath Nandennavar ◽  
Veerendra Angadi
Keyword(s):  
Treatment Outcomes ◽  
Retrospective Analysis ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Single System ◽  
Multisystem Disease ◽  
Cancer Hospital ◽  
Mucosal Involvement

Background: Langerhans cell histiocytosis (LCH) comprises a diverse group of disorders where pathologic Langerhans cells accumulate in a variety of organs. Aims and objectives of the study is to analyse the clinical manifestations and treatment outcomes of patients diagnosed with LCH in a tertiary cancer hospital in South India.Methods: Retrospective analysis of the case records of patients presenting with histological proven case of LCH over a period of 7 years from 2011 to 2018, being treated at Vydehi Institute of Medical Sciences and Research Centre.Results: 10 patients with biopsy proven LCH were included. The median age of diagnosis was 8 years (range 1 to 73 years) and 3 patients aged 18 years or older at the time of diagnosis. The male: female ratio was 3:2. Multisystem involvement was found in 4 patients (40%) and Single system Involvement in remaining 6 patients. Isolated bone lesions were found in 4 patients (40%), 1 patient had isolated Lymph node involvement; 1 patient had oral cavity lesion. None of the 4 patients with multisystem diseases had skin/mucosal involvement; 3 had bony involvement, 2 patients had lung involvement. One patients with multisystem disease expired while 5 patients were lost to follow-up. 4 out of the 10 patients are on regular follow-up and are in remission.Conclusions: Despite limitation by the retrospective nature, this descriptive study was done to provide further disease information regarding Indian population. Data from this study clearly confirms the known fact that most of the patients with Single System LCH have a very good response rate. Patients with multisystem disease have the highest risk of disease related mortality and morbidity as one among the 4 patients with multisystem disease died just after initiating treatment.

Clinical course of the bony lesion of single-system single-site Langerhans cell histiocytosis – Is appropriate follow-up sufficient treatment?

2018 ◽  
Vol 23 (1) ◽  
pp. 168-173 ◽  
Author(s):  
Hiromi Sasaki ◽  
Satoshi Nagano ◽  
Hirofumi Shimada ◽  
Shunsuke Nakamura ◽  
Takao Setoguchi ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Course ◽  
Langerhans Cell ◽  
Single Site ◽  
Single System ◽  
Bony Lesion

scholarly journals LANGERHANS CELL HISTIOCYTOSIS OF THE SCAPULA - DIAGNOSIS & TREATMENT OPTIONS

2017 ◽  
Vol 16 (3) ◽  
pp. 240-243 ◽  
Author(s):  
ROHIT PANDEY ◽  
HIMANSHU BHAYANA ◽  
RAJESH KUMAR RAJNEESH ◽  
ISH KUMAR DHAMMI ◽  
REHAN UL-HAQ ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Langerhans Cell ◽  
Single System ◽  
Diagnostic Dilemma ◽  
Systemic Involvement ◽  
System Disease ◽  
S 100 ◽  
Specific Finding

ABSTRACT Langerhans cell histiocytosis (LCH) is characterised by an abnormal histiocytic accumulation in tissues such as the lung, spleen, bone marrow, skin, central nervous system, liver and lymph nodes, causing focal or systemic effects. No specific clinical & radiographic presentation of LCH is described in literature. This poses a diagnostic dilemma for surgeons. The scapula is the site of 3% of bone tumours, while for LCH it is the least common site. In a 10-year-old boy with isolated lesion of the scapula with no other systemic involvement, and no specific finding in MRI or CT scan of scapula, diagnosis was confirmed on biopsy. Division into single and multi-system disease is paramount in treatment, given that it is a single system disease. The patient improved clinically on follow-up of 2 years. The scapula is one of the rarest site of LCH, and because various lesions mimic each other, a biopsy is always required, with immunohistochemistry for CD68 & S-100. This was only a single system disease, so conservative management was performed, and the patent improved clinically.

PP – 10-YEAR FOLLOW- UP OF A SINGLE-SYSTEM LANGERHANS CELL HISTIOCYTOSIS - MULTIFOCAL BONE INVOLVEMENT

2017 ◽  
Vol 123 (2) ◽  
pp. e66
Author(s):  
RENATA MENDONÇA MORAES ◽  
JOYCE GIMENEZ MENON ◽  
JULIANA ROCHA VERRONE ◽  
JOSÉ DIVALDO PRADO ◽  
FERNANDO AUGUSTO SOARES ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Bone Involvement ◽  
Single System

scholarly journals Langerhans-Cell Histiocytoses - Epidemiology, Classification, Clinical Features, Diagnosis, Complications, Treatment and Prognosis

2016 ◽  
Vol 9 (1) ◽  
pp. 3-16 ◽  
Author(s):  
Vera E. Papochieva ◽  
Dimitrinka S. Miteva ◽  
Penka I. Perenovska ◽  
Guergana Petrova
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Eosinophilic Granuloma ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
Multisystem Disease ◽  
The Past ◽  
System Disease ◽  
Young Smokers ◽  
Underlying Pathology

Summary Histiocytoses comprise a group of diverse diseases of unknown etiology with various clinical presentation and evolution. The underlying pathology is characterised by accumulation and infiltration of variable numbers of cells of the monocyte-macrophage line in the affected tissues and organs. Histiocytoses are divided into three major classes: Langerhans cell histiocytosis (LCH), non- Langerhans cell histiocytosis, and malignant histiocytic disorders. The term LCH (also known in the past as histiocytosis X) encompasses the following rare diseases: Eosinophilic Granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, Hashimoto-Pritzker disease, in which accumulation of pathologic Langerhans cells (LCs) leads to tissue damage. LCs usually reside in the skin and ensure protection against infections by destroying foreign substances. LC accumulation is caused by antigen stimulation and inadequate immune response. Thus, clinical LCH manifestations range from isolated disease with mono- or multifocal bone lesions to disseminated multisystem disease. LCH is a rare disease, affecting mainly children and young smokers, aged 20-50 years. Lung involvement in LCH usually presents as a mono-system disease and is characterized by Langerhans cell granulomas (LCG) infiltrating and impairing the distal bronchioles. The definite diagnosis is based on lung biopsy of CAT selected LCG areas. So far, there is no an effective treatment, but the better understanding of the mechanisms involved in the pathogenesis of the disease would help in the development of effective therapeutic strategies in the future.

Adult Langerhans cell histiocytosis: A contemporary single-institution series of 186 patients.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 7018-7018
Author(s):  
Gaurav Goyal ◽  
Marie Hu ◽  
Jason R Young ◽  
Robert Vassallo ◽  
Jay H Ryu ◽  
...  
Keyword(s):  
Smoking Cessation ◽  
Langerhans Cell Histiocytosis ◽  
Overall Response Rate ◽  
Langerhans Cell ◽  
Braf V600e ◽  
First Line ◽  
Multisystem Disease ◽  
Presenting Symptoms ◽  
Systemic Therapies

7018 Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm driven by MAPK-ERK mutations in majority of patients. Contemporary data on treatments and outcomes in adult LCH are lacking. Hence, we undertook this study to analyze a large cohort of adult LCH patients. Methods: This was a retrospective study of adult (≥18 years) LCH patients seen at our institution between 1998 and 2018. Results: We included 186 patients with adult LCH (median age 43; 19-88), and 54% were females. 70% of patients were diagnosed after 2007. Common presenting symptoms were cough/dyspnea (30%), rash (17%), pain/swelling in head (17%), and diabetes insipidus (10%). 70 (38%) patients had multisystem LCH, 62 (33%) had isolated pulmonary LCH, and 35 (19%) had unifocal LCH. Common sites of involvement included lung (59%), bone (37%), skin (21%), and nervous system (16%). 121 (65%) were smokers; 48% of these had lung disease, while 52% had multisystem disease. 18 of 31 tested (58%) patients had BRAF-V600E mutation. Most common first-line treatment was smoking cessation in 24 patients, and led to an overall response rate (ORR) of 83% in pulmonary lesions. Radiation therapy was used in 11 patients, and led to an ORR 82%. Surgical resection of lesion was done in 23 patients, with relapses in 24%. Systemic therapies were used in 78 (42%) patients (Table). Most common first-line systemic therapy was cladribine with ORR of 78%. Vemurafenib was used in 3 patients with BRAF-V600E, leading to an ORR of 67% . After a median follow-up of 23 months (0-261), 21 patients had died. Of these, 10 died of progressive LCH. Median OS was not reached, and mean OS was 196 months. Conclusions: This is the largest contemporary series of adult LCH. It shows that diverse clinical spectrum, ranging from benign course to a progressive multisystem disease. Although smoking cessation was an effective treatment for pulmonary LCH, a large subset required systemic chemotherapy. [Table: see text]

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