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.

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.

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

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]

scholarly journals Response to immunomodulatory drug-based regimens in heavily pretreated patients with recurrent/refractory adult Langerhans cell histiocytosis

2019 ◽  
Author(s):  
Xin-xin Cao ◽  
Ai-lin Zhao ◽  
Xue-min Gao ◽  
Hui-lei Miao ◽  
Hong-xiao Han ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Median Number ◽  
Langerhans Cell ◽  
Medical College ◽  
Multisystem Involvement ◽  
Heavily Pretreated ◽  
Pretreated Patients ◽  
Disease Reactivation ◽  
Thalidomide Treatment

Abstract Purpose: Langerhans cell histiocytosis (LCH) is a clonal histiocytic neoplasm and, because of its rarity in adults, there is no standard treatment for adult LCH. Immunomodulatory drugs (IMiDs) have been used to treat patients with low risk recurrent/refractory LCH but their effectiveness in adult LCH patients is unclear.Methods: We retrospectively evaluated the response rate to IMiDs-based regimens in ten heavily pretreated recurrent/refractory adult LCH patients at Peking Union Medical College Hospital.Results: A total of 10 patients (four males and six females) were included in this study. Median age at diagnosis was 33 years (range, 28–54 years). All patients had multisystem involvement and the median number of organs involved was 5 (range, 5–7). Seven patients had high risk organs involved, including seven patients with liver involvement and one with spleen involvement. All 10 patients had received at least one chemotherapy before the IMiDs-based regimen. The median number of previous lines of chemotherapy was 2 (range, 1–4). Eight patients received thalidomide, dexamethasone and cyclophosphamide, and two patients received lenalidomide and dexamethasone. The median time that patients received thalidomide treatment was 15 months and the duration of the two patients on lenalidomide regimen was 3 months and 12 months separately. Treatment responses in eight recurrent LCH patients included non-active disease in one patient and regressive disease in seven patients. The two refractory patients who had progression during the last treatment had stable disease after IMiDs therapy. During a median 15-months follow-up period, no disease reactivation or death was observed.Conclusions: IMiDs combined with dexamethasone and cyclophosphamide, may be a salvage therapy for recurrent/refractory adult LCH patients.

BRAFV600E frequency and impact on outcomes in adults with langerhans cell histiocytosis.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 7050-7050
Author(s):  
Aldo A. Acosta-Medina ◽  
Jithma P. Abeykoon ◽  
N. Nora Bennani ◽  
Caroline Davidge-Pitts ◽  
Matthew J. Koster ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Disease Risk ◽  
Clinical Manifestations ◽  
Braf Inhibitor ◽  
Progression Free Survival ◽  
Langerhans Cell ◽  
Rank Test ◽  
Multisystem Disease ◽  
Mutational Status ◽  
Increased Risk

7050 Background: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm manifesting as unifocal, multifocal, multisystem (MS) or pulmonary LCH (smoking-related). In pediatric LCH, somatic BRAFV600E prevalence is reported at 55-70%, and associated with increased risk of multisystem disease and early treatment failure. Our aim was to describe the prevalence of BRAFV600E mutation and evaluate its association with clinical manifestations and outcomes in adults with LCH. Methods: A retrospective review of adult patients diagnosed with LCH consecutively seen at Mayo Clinic from 2011 to 2020 was performed.Evaluation of association of BRAFV600E mutational status and clinical factors was conducted by the Chi-square test for independence. Progression-free survival (PFS) and overall survival (OS) were analyzed via the Kaplan Meier method and compared with the log-rank test to assess the effect of BRAFV600E. Results: Of the total LCH cohort ( n= 128), 88 patients with available BRAFV600E results were included in the study. Median age at diagnosis was 41y (range 19 - 88); 52.3% were male. 40 (45.5%) patients had a BRAFV600E mutation. Increasing age was associated with BRAFV600E (10-year increase OR 1.42, 95%CI 1.07-1.89; p= 0.017). No correlation was observed between BRAFV600E status and site of disease, risk organ (RO: liver, spleen, marrow) involvement, or MS disease. Patients with BRAFV600E were 4 times more likely to receive targeted therapy ( BRAF inhibitor) than non- BRAFV600E patients ( p= 0.018). After a median follow up of 46 mo (95% CI 30.8-61.2), PFS was similar between BRAFV600E and non- BRAFV600E patients ( p= 0.167). However, patients with BRAFV600E had a worse 3-year OS compared with non- BRAFV600E patients (84% vs. 97.1%, p= 0.027). Patients who died had a significantly higher age at LCH diagnosis (median 62 vs. 38 years; p= 0.0002). Conclusions: In our cohort of adults with LCH, BRAFV600E was less frequent than reported in pediatric literature and was associated with worse OS. The frequency of BRAFV600E was positively correlated with increasing age. Contrary to reports in pediatric LCH, there were no significant associations between BRAFV600E and high-risk or multisystem disease.[Table: see text]

scholarly journals Adult Langerhans cell histiocytosis presenting with multisystem involvement and sarcomatoid features: a case report

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Luis E. Aguirre ◽  
Ingrid Schwartz ◽  
Jennifer Chapman ◽  
Marcelo F. Larsen ◽  
Alvaro Alencar
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Poor Prognosis ◽  
Langerhans Cell ◽  
Multisystem Disease ◽  
Cell Sarcoma ◽  
Langerhans Cell Sarcoma ◽  
Disease Biology ◽  
Multisystem Involvement ◽  
Acute Monoblastic Leukemia ◽  
Poor Outcomes

Abstract Background Langerhans cell tumors are rare clonal disorders characterized by neoplastic proliferation of dendritic cells that can be further classified into the subtypes Langerhans cell histiocytosis and Langerhans cell sarcoma, which are rare neoplasms exhibiting aggressive features and a poor prognosis. In addition to illustrating the refractoriness and poor outcomes of multisystem Langerhans cell histiocytosis in adults, specific events in this case highlight important characteristics of disease biology that warrant detailed discussion and exposition to a wider audience. Case presentation We describe the case of a 42-year-old Caucasian man with Langerhans cell histiocytosis diagnosed from a lesion on the left arm that presented with constitutional symptoms, early satiety, and weight loss. Esophagogastroduodenoscopy showed extensive esophageal and duodenal involvement by Langerhans cell histiocytosis with features of Langerhans cell sarcoma. He was initially treated for Langerhans cell histiocytosis with low doses of cytarabine until he eventually presented clear transformation to acute monoblastic leukemia with complex karyotype that could not be properly controlled, leading eventually to death. Conclusions Langerhans cell histiocytosis remains an exceedingly rare entity in adults, frequently presenting as multisystem disease with risk organ involvement. Langerhans cell sarcoma represents an aggressive subtype with extremely poor prognosis for which intensive acute myeloid leukemia induction should be strongly considered.

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

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