scholarly journals How I treat Langerhans cell histiocytosis

Blood ◽  
2015 ◽  
Vol 126 (1) ◽  
pp. 26-35 ◽  
Author(s):  
Carl E. Allen ◽  
Stephan Ladisch ◽  
Kenneth L. McClain
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Randomized Clinical Trials ◽  
Bone Lesion ◽  
Disease Recurrence ◽  
Langerhans Cell ◽  
Low Risk ◽  
Clinical Severity ◽  
Mitogen Activated Protein Kinase ◽  
Risk Patients ◽  
Single Bone

Abstract “Langerhans cell histiocytosis” (LCH) describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Regardless of clinical severity, LCH lesions share the common histology of CD1a+/CD207+ dendritic cells with characteristic morphology among an inflammatory infiltrate. Despite historical uncertainty defining LCH as inflammatory vs neoplastic and incomplete understanding of mechanisms of pathogenesis, clinical outcomes have improved markedly over the past decades through cooperative randomized clinical trials based on empiric therapeutic strategies. Significant advances include recognition of high- and low-risk clinical groups defined by hematopoietic and/or hepatic involvement, and of the importance of optimal intensity and of duration of chemotherapy. Nevertheless, mortality of high-risk patients, disease recurrence, lack of robustly tested salvage strategies, and significant disease morbidity of both high- and low-risk patients remain challenges. Recent discovery of recurrent somatic mutations in mitogen-activated protein kinase pathway genes at critical stages of myeloid hematopoietic differentiation in LCH patients supports redefinition of the disease as a myeloproliferative disorder and provides opportunities to develop novel approaches to diagnosis and therapy.

scholarly journals Langerhans Cell Histiocytosis as Cause of Central Diabetes Insipidus: Case Report

2018 ◽  
Vol 37 (01) ◽  
pp. 76-79 ◽  
Author(s):  
Diana Nascimento ◽  
Manoel Teixeira ◽  
Eberval Figueiredo
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Vital Functions ◽  
Hypothalamic Pituitary Axis ◽  
The Individual ◽  
Single Bone ◽  
Made In

AbstractLangerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system, characterized by the aberrant proliferation of specific dendritic cells. The clinical presentation ranges from a single bone lesion to widespread multiorgan involvement. This disease is usually considered to be a disease of childhood; however, the diagnosis is frequently made in adulthood. The course of the disease is fairly unpredictable and varies from spontaneous resolution to progress into a debilitating form, which compromises the vital functions with occasional fatal consequences. Langerhans cell histiocytosis exhibits a predilection for the hypothalamic-pituitary-axis, with diabetes insipidus being the most common endocrine consequence related to the disease, which may be prior to diagnosis or develop at any time during the course of the disease. The diagnosis of LCH should be based on histologic and immunophenotypic examination of a lesional biopsy, although other testing may be done, depending on the symptoms. There is no established, widely agreed-upon treatment of LCH, in general. The treatment depends upon the individual patient and the extent and areas of involvement. The present article aims to describe the case of a 26-year-old male patient whose symptoms started with a headache and occipital bone lesion that progressed later with diabetes insipidus.

scholarly journals Treatment of Multifocal Multisystem BRAF Positive Langerhans Cell Histiocytosis with Cladribine, Surgery and Allogenic Stem Cell Transplantation

2017 ◽  
Vol 60 (4) ◽  
pp. 152-156 ◽  
Author(s):  
Filip Gabalec ◽  
Martin Šimkovič ◽  
Alžběta Zavřelová ◽  
Petra Kašparová ◽  
Paula Morávková ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Allogeneic Stem Cell Transplantation ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Allogenic Stem Cell Transplantation ◽  
Single Bone

Langerhans cell histiocytosis (LCH) is a very rare disease in adults and as well a very rare cause of sellar expansion. The clinical presentation can be heterogeneous, from a single bone lesion to potentially fatal, widespread disease. We describe the difficulties with the diagnosis and treatment of LCH as well as successful treatment with cladribine chemotherapy and allogeneic stem cell transplantation.

scholarly journals Solitary tibial lesion as the initial presentation of Langerhans cell histiocytosis: report of two cases and literature review

2021 ◽  
Vol 49 (1) ◽  
pp. 030006052098282
Author(s):  
Chih-Yang Lin ◽  
Chia-Che Lee ◽  
Kuan-Wen Wu ◽  
Chang-Tsu Yuan ◽  
Ken-Nan Kuo ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Langerhans Cell Histiocytosis ◽  
Paediatric Oncology ◽  
Bone Diseases ◽  
Langerhans Cell ◽  
Oncology Group ◽  
Preventive Chemotherapy ◽  
System Risk ◽  
Risk Patients

The various presentations of osseous Langerhans cell histiocytosis (LCH) make it difficult to distinguish from other bone diseases. In addition, there is no universally accepted protocol for managing osseous LCH for single non-central nervous system-risk lesions. Here, the rare cases of two paediatric patients, aged 1 and 2 years, who presented with a solitary tibial lesion at time of LCH diagnosis, are reported. One patient progressed to multiple lesions after curettage of the original lesion. Subsequently, both patients received preventive chemotherapy using the Taiwan Paediatric Oncology Group (TPOG) revised protocol for treating low risk patients with LCH, namely, TPOG LCH2002-LR. After receiving this treatment, which included a schedule of prednisolone and vincristine for 6 weeks, followed by prednisolone, vincristine and 6-mercaptopurine for a further 48 weeks, both patients are free from recurrence or progression.

Isolated Langerhans Cell Histiocytosis Bone Lesion in Pediatric Patients

2015 ◽  
Vol 153 (5) ◽  
pp. 751-757 ◽  
Author(s):  
Aren Bezdjian ◽  
Abdullah A. Alarfaj ◽  
Namrata Varma ◽  
Sam J. Daniel
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Bone Lesion ◽  
Langerhans Cell

Pulmonary Langerhans Cell Histiocytosis

2020 ◽  
Vol 41 (02) ◽  
pp. 269-279
Author(s):  
Brian Shaw ◽  
Michael Borchers ◽  
Dani Zander ◽  
Nishant Gupta
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Myeloid Neoplasm ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Mitogen Activated Protein ◽  
The Central Nervous System

AbstractPulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. A subset of patients with PLCH may have extrapulmonary involvement, typically involving the skeletal system in the form of lytic lesions, skin lesions, or the central nervous system most commonly manifesting in the form of diabetes insipidus. Smoking cessation is the cornerstone of treatment in patients with PLCH and can lead to disease regression or stabilization in a substantial proportion of patients. Further insight into the underlying molecular pathogenesis of PLCH has paved the way for the future development of disease-specific biomarkers and targeted treatment options directed against the central disease-driving mutations.

Zoledronic acid for relapsed Langerhans cell histiocytosis with isolated skull bone lesion

2019 ◽  
Vol 61 (3) ◽  
pp. 315-317 ◽  
Author(s):  
Ko Kudo ◽  
Tatsuhiko Tanaka ◽  
Akie Kobayashi ◽  
Kiminori Terui ◽  
Etsuro Ito
Keyword(s):  
Zoledronic Acid ◽  
Langerhans Cell Histiocytosis ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Skull Bone

TNF-α-238GA, IL-1α-889CC and IL-10–819CC Polymorphisms Confere an Increased Risk to Develop Multisystem Langerhans Cell Histiocytosis.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1331-1331
Author(s):  
Paola De Filippi ◽  
Cesare Danesino ◽  
Diego Ferrarese ◽  
Annalisa De Silvestri ◽  
Carla Badulli ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Bone Lesion ◽  
Multiple Organ ◽  
Langerhans Cell ◽  
Cytokine Network ◽  
Regional Lymph Nodes ◽  
Cytokines And Chemokines ◽  
Increased Risk ◽  
Tnf Α ◽  
Disseminated Disease

Abstract Langerhans cell Histiocytosis (LCH) (OMIM 604856) is a rare disorder affecting any age, with a highly variable clinical course, usually related to the number and type of organs affected at the time of presentation. The symptoms range from a solitary bone lesion that does not require treatment to a disseminated disease with multiple-organ involvement and a high mortality rate, despite aggressive treatment. The pathogenesis of LCH remains unclear, although deregulated growth, activity and trafficking of Langerhans cells (LC) are implicated. The ability of LC to migrate from the epidermis to regional lymph nodes is of pivotal importance for the induction of immune response and there is increasing evidence that both cytokines and chemokines are implicated in this function. We report the analysis of polymorphisms in genes coding for different cytokines in a population of patients with LCH in comparison to an Italian control population (n=140). We studied 40 patients, with a mean age at the disease of onset of 6.1 (±5.0) years; the 15 with single-system (SS) disease had a mean age of 7.1 (±3.9) years, while the 25 with multi-system (MS) disease had a mean age of 5.3 (±5.3) years. The Cytokine Genotyping Tray (Pel-Freez, Milwaukee, USA) was used for the detection of the following polymorphisms: Il-1α-889 C/T, Il-1β (−511 C/T; +3962 T/C), IL-1R pst1 1970 C/T, IL-RA mspa1 11100 T/C, IL-4Rα +1902 G/A, IL-12 -1188 C/A, γ IFN UTR 5644 A/T, TNF α (−308 G/A; −238 G/A), IL-2 (−330 T/G; +160 G/T), IL-4 (−1098T/G; −590 T/C; −33 T/C), IL-6 (−174 G/C; nt565 G/A), IL-10 (−1082 G/A; −819 C/T; −592 C/A). The following polymorphisms showed a different distribution in patients versus controls: IL-4–33 (p=0.048); IL-4–590 (p=0.005); IL-1α-889 (p=0.009). The correspondence analysis of these variables showed that genotypes IL-4 -33TT, IL-4 -590 TC and-1β+3962TT confer an increased risk of developing SS disease, while genotypes TNF-α-238GA, IL-1α-889CC and IL-10–819CC give an increased risk of developing MS disease. The present study supports the concept that constitutional variants affecting the cytokine network may induce susceptibility to develop LCH and also affect its manifestations. Figure Figure

Treatment of recurrent Langerhans cell histiocytosis of the vulva with lenalidomide.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e16555-e16555 ◽  
Author(s):  
Ibrahim Fuad Ibrahim ◽  
Harris V. K. Naina
Keyword(s):  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Symptom Control ◽  
Female Genital Tract ◽  
Disease Recurrence ◽  
Langerhans Cell ◽  
Effective Treatment Option ◽  
Distant Disease ◽  
First Line Therapy

e16555 Background: Langerhans cell histiocytosis (LCH) of the female genital tract is an extremely rare disease. We report a 14 year follow up of a patient with LCH successfully treated with lenalidomide in the relapsed setting. Methods: N/A. Results: A 43 year old Ethiopian woman initially presented to another institution with a nodular lesion on her left vulva with biopsy consistent with LCH. Metastatic workup at the time did not reveal evidence of distant disease. The patient was initially treated with radiotherapy to the vulva and 2 years later she was diagnosed with recurrent disease in the vulva and underwent a wide local excision. Six months afterwards she developed a lesion on her right labium majus consistent with disease recurrence. She was treated with radiotherapy again and underwent a wide radical vulvar excision. She recurred only 3 months later and was started on salvage therapy with thalidomide, 3 years after the initial diagnosis. Within 2 months of starting thalidomide therapy, the patient experienced resolution of vulvar lesions and symptoms. She remained symptom free for 8 years while on thalidomide but then presented to our institution with a new central vulvar lesion after being off therapy for 4 months. Vulvar biopsy confirmed the presence of LCH. She was subsequently restarted on thalidomide and achieved symptom control for an additional 18 months before developing worsening pain and neuropathy associated with therapy. Imaging revealed no evidence of metastatic disease. We then started lenalidomide therapy at dose of 10 mg daily for 21 days in a 28 day cycle and subsequently increased the dose to 25 mg daily continuously. She achieved marked improvement in vulvar symptoms within a month of initiation of lenalidomide. She has tolerated therapy well with no neuropathy or cytopenias. Conclusions: Primary vulvar LCH is a rare disease with no standard therapies. Thalidomide has been described in the literature as an effective treatment option. We describe the use of lenalidomide as an alternative and well tolerated therapy in the relapsed setting. Further investigation is required to determine whether lenalidomide is a viable first line therapy for this rare disease.

Correlative study of disease activity in Langerhans cell histiocytosis.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e19540-e19540
Author(s):  
Jeffrey Garnet Edwards ◽  
Michael Raymond Jeng
Keyword(s):  
Alkaline Phosphatase ◽  
Logistic Regression ◽  
Disease Activity ◽  
Langerhans Cell Histiocytosis ◽  
Subjective Evaluation ◽  
Disease Status ◽  
Disease Recurrence ◽  
Current Treatment ◽  
Langerhans Cell ◽  
System Involvement

e19540 Background: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia that primarily affects younger children, most often involving bone, skin, lung, lymph nodes, or liver. Current treatment for LCH includes chemotherapy and biologically targeted therapies. Given its clinical heterogeneity and low prevalence, decision-making regarding intervention relies on subjective evaluation of disease status, which is often dependent on provider experience. Thus, there is a need to identify biomarkers for disease recurrence and activity in patients with LCH. Methods: We conducted a retrospective cohort study including pediatric LCH patients (n = 48) ≤21 years of age with biopsy-proven diagnosis of LCH and ≥1 year of follow-up after diagnosis. Data regarding demographics, symptom history, organ system involvement, and laboratory values at different time points were extracted from the from patient medical records and entered into a REDCap Database. The primary outcome was recurrence status as measured by the end of the study period. Data were analyzed using Stata statistical analysis software. Results: In our cohort, the sample was 64.6% Male and 35.4% Female with a median age of 29 months at diagnosis. 68.1% of patients had single organ involvement while 31.9% exhibited multi-system involvement. 70.8% of our cohort received treatment with chemotherapy with an average initial treatment duration of 12.1 months. 27.1% of the cohort experienced recurrence by the time of chart review. Logistic regression of the demographic factors and lab values at diagnosis revealed a statistically significant association between multi-system involvement (OR: 3.94, p = 0.04), moderate or high central nervous system risk assessment (OR: 7.67, p = 0.03; OR: 5.75, p = 0.03) and likelihood of recurrence. Additionally, logistic regression analysis revealed that alkaline phosphatase level at the time of diagnosis was associated with a slightly decreased likelihood of recurrence (OR: 0.987, p = 0.02). Conclusions: These results reveal that in addition to the traditionally utilized inflammatory markers (Erythrocyte Sedimentation Rate, White Blood Cell and Platelet Count), Alkaline Phosphatase could be used as a predictor of disease activity for patients with LCH, especially for those with bone involvement. Moving forward, an increased sample size might reveal other correlative biomarkers that can predict disease activity, helping to more precisely manage LCH.

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