scholarly journals Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Bruno Araujo ◽  
Francisco Costa ◽  
Joanne Lopes ◽  
Ricardo Castro
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Early Stage ◽  
Biliary Tree ◽  
Pulmonary Involvement ◽  
Multiple Organ ◽  
Langerhans Cell ◽  
Liver Involvement ◽  
Unknown Etiology ◽  
Multisystem Disease ◽  
Organ Systems

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.

scholarly journals Langerhans Cell Histiocytosis: a Case Report

2013 ◽  
Vol 5 (2) ◽  
pp. 74-86
Author(s):  
Mirjana Paravina ◽  
Dragan Jovanović ◽  
Milenko Stanojević ◽  
Ljiljana Nikolić
Keyword(s):  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Early Stage ◽  
Fatal Outcome ◽  
Langerhans Cell ◽  
Neoplastic Disease ◽  
Multisystem Disease ◽  
Organ Systems ◽  
The Right

Abstract Langerhans cell histiocytosis is a disease which results from accumulation or proliferation of a clonal population of cells with the phenotype of Langerhans cells arrested at an early stage of activation that are functionally deficient. The etiology and pathogenesis of the disorder are still unknown. There are ongoing investigations to determine whether it is a reactive or a neoplastic disease. The fact is that neoplastic and reactive processes may have many clinical and pathological similarities. Some emphasize the role of “cytokine storm” in Langerhans cells. Further studies are necessary in all areas, from the etiology and pathogenesis to diagnosis and therapy. Langerhans cell histiocytosis primarily affects bones, but less commonly it may involve other organ systems, or present as a multisystem disease. The clinical course is variable, from benign forms with spontaneous resolution, to chronic disseminated forms with fatal outcome. This is a report of a 29-year-old man with Langerhans cell histiocytosis with an onset at the age of 8, which later progressed to a multisystem disease. Apart from lesions on the skin and exposed mucous membranes, the patient also presented with: diabetes insipidus, granuloma of the right femur and slight bulbar protrusion of the right eye. The patient experienced spontaneous pneumothorax on two occasions. The diagnosis of Langerhans cell histiocytosis was histologically confirmed using electron microscopy by presence of Birbeck granules in the histiocytes. A favorable therapeutic response was obtained after systemic corticosteroid therapy.

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.

scholarly journals Langerhans cell histiocytosis of liver

2020 ◽  
Vol 8 (8) ◽  
pp. 3079
Author(s):  
Swapna Samudrala ◽  
Anuradha Sekaran ◽  
Bharat Patodiya ◽  
Nageshwar Reddy Duvuru
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Survival Rates ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Hepatic Involvement ◽  
Organ Systems ◽  
Impact On Survival ◽  
Considerable Impact ◽  
Single Organ

Langerhans cell histiocytosis (LCH) is a rare disorder of unknown etiology caused by proliferation of Langerhans cells. It can involve single organ system to multi organ systems and clinical presentation is variable depending on the organ involved and have different prognosis. LCH is common in children when compared to adults. Hepatic involvement in adults is relatively rare. Liver involvement has considerable impact on survival rates. Histopathology and immunohistochemistry provide the definitive diagnosis. Authors report a case of Langerhans cell histiocytosis in a young adult with hepatic involvement.

scholarly journals Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
K. Earlam ◽  
C. A. Souza ◽  
R. Glikstein ◽  
M. M. Gomes ◽  
S. Pakhalé
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pulmonary Involvement ◽  
Langerhans Cell ◽  
Cytotoxic Agents ◽  
Current Cigarette Smoking ◽  
System Disease ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Multisystem Involvement ◽  
Organ Systems ◽  
Current Cigarette

Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

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 Paediatric Langerhans Cell Histiocytosis Disease: Long-Term Sequelae in the Hypothalamic Endocrine System

2021 ◽  
pp. 1-9
Author(s):  
Elisa Vaiani ◽  
Guido Felizzia ◽  
Fabiana Lubieniecki ◽  
Jorge Braier ◽  
Alicia Belgorosky
Keyword(s):  
Anterior Pituitary ◽  
Langerhans Cell Histiocytosis ◽  
Endocrine System ◽  
Langerhans Cell ◽  
Hormone Deficiency ◽  
Bone Lesions ◽  
Pituitary Hormone ◽  
Multisystem Disease ◽  
Anterior Pituitary Hormone

Langerhans cell histiocytosis (LCH) is a disorder of the mononuclear phagocyte system that can affect almost any organ and system. The most common central nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitary region leading to destruction and neurodegeneration of CNS tissue. The latter causes the most frequent endocrinological manifestation, that is, central diabetes insipidus (CDI), and less often anterior pituitary hormone deficiency (APD). The reported incidence of CDI is estimated between 11.5 and 24% and is considered a risk factor for neurodegenerative disease and APD. Three risk factors for development of CDI are recognized in the majority of the studies: (1) multisystem disease, (2) the occurrence of reactivations or active disease for a prolonged period, and (3) the presence of craniofacial bone lesions. Since CDI may occur as the first manifestation of LCH, differential diagnosis of malignant diseases like germ cell tumours must be made. APD is almost always associated with CDI and can appear several years after the diagnosis of CDI. Growth hormone is the most commonly affected anterior pituitary hormone. Despite significant advances in the knowledge of LCH in recent years, little progress has been made in preventing long-term sequelae such as those affecting the hypothalamic-pituitary system.

Pulmonary Langerhans Cell Histiocytosis and Other Pulmonary Histiocytic Diseases: A Review

2008 ◽  
Vol 132 (7) ◽  
pp. 1171-1181 ◽  
Author(s):  
Timothy Craig Allen
Keyword(s):  
Literature Review ◽  
Langerhans Cell Histiocytosis ◽  
Correct Diagnosis ◽  
Pulmonary Involvement ◽  
Langerhans Cell ◽  
Data Sources ◽  
Primary Material ◽  
Pulmonary Langerhans Cell Histiocytosis

Abstract Context.—Pulmonary Langerhans cell histiocytosis is the most common and best known pulmonary histocytic lesion; however, the realm of pulmonary histiocytic lesions also includes an assortment of uncommon diseases that may exhibit pulmonary involvement. Objective.—To review pulmonary Langerhans cell histiocytosis and other pulmonary histiocytoses to better ensure correct diagnosis and optimal assessment of prognosis and treatment. Data Sources.—Literature review and primary material from the author's institution. Conclusions.—This review discusses the most common pulmonary histocytosis, pulmonary Langerhans cell histiocytosis, and also reviews the uncommon pulmonary histiocytic lesions, which are distinct from pulmonary Langerhans cell histiocytosis.

Adult-onset Neurological Langerhans Cell histiocytosis and Response to Treatment

2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Al-Hader R ◽  
◽  
Suneja A ◽  
Memon AB ◽  
Mukherje A ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Brain Magnetic Resonance Imaging ◽  
Langerhans Cell ◽  
Response To Therapy ◽  
Response To Treatment ◽  
Adult Onset ◽  
Multisystem Disease ◽  
Clonal Proliferation ◽  
Magnetic Resonance Imaging Mri ◽  
Mass Lesions

Introduction: Langerhans Cell Histiocytosis (LCH) is a rare form of cancer that mostly affects children and rarely adults. LCH involves an abnormal clonal proliferation of Langerhans cells in the bone marrow. These cells are capable of migrating from the skin to lymph nodes. Therefore, it is characterized as a multisystem disease. Neurological manifestations are not common, and often patients’ present with endocrine dysfunction with neuroimaging findings of hypothalamic and pituitary masses can mimic pituitary adenoma. Here, we discuss two instances of unusual adult-onset, primary neurological LCH in patients with a positive response to therapy-these two patients presented with mass lesion and neurodegenerative form of LCH, respectively. LCH can manifest features of mass lesions or neurodegeneration on brain Magnetic Resonance Imaging (MRI). Since it is rare in adults, it is crucial to identify this condition as timely treatment can have a better prognosis.

scholarly journals Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Solenne Le Louet ◽  
Mohamed-Aziz Barkaoui ◽  
Jean Miron ◽  
Claire Galambrun ◽  
Nathalie Aladjidi ◽  
...  
Keyword(s):  
Targeted Therapy ◽  
Langerhans Cell Histiocytosis ◽  
Treatment Guidelines ◽  
Langerhans Cell ◽  
Infection Prophylaxis ◽  
Lung Involvement ◽  
Multisystem Disease ◽  
Abnormal Chest ◽  
Life Threatening ◽  
Severe Lung

Abstract Background Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed. Methods Among 1482 children (< 15 years) registered in the French LCH registry (1994–2018), 111 (7.4%) had lung involvement. This retrospective study included data for 17 (1.1%) patients that required one or more intensive care unit (ICU) admissions for respiratory failure. Results The median age was 1.3 years at the first ICU hospitalization. Of the 17 patients, 14 presented with lung involvement at the LCH diagnosis, and 7 patients (41%) had concomitant involvement of risk-organ (hematologic, spleen, or liver). Thirty-five ICU hospitalizations were analysed. Among these, 22 (63%) were secondary to a pneumothorax, 5 (14%) were associated with important cystic lesions without pneumothorax, and 8 (23%) included a diffuse micronodular lung infiltration in the context of multisystem disease. First-line vinblastine–corticosteroid combination therapy was administered to 16 patients; 12 patients required a second-line therapy (cladribine: n = 7; etoposide-aracytine: n = 3; targeted therapy n = 2). A total of 6 children (35%) died (repeated pneumothorax: n = 3; diffuse micronodular lung infiltration in the context of multisystem disease: n = 2; following lung transplantation: n = 1). For survivors, the median follow-up after ICU was 11.2 years. Among these, 9 patients remain asymptomatic despite abnormal chest imaging. Conclusions Severe lung involvement is unusual in childhood LCH, but it is associated with high mortality. Treatment guidelines should be improved for this group of patients: viral infection prophylaxis and early administration of a new LCH therapy, such as targeted therapy.

scholarly journals Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Rashid AL Umairi ◽  
Danielle Blunt ◽  
Wedad Hana ◽  
Matthew Cheung ◽  
Anastasia Oikonomou
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Corticosteroid Treatment ◽  
Pulmonary Involvement ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Sinus Histiocytosis ◽  
Review Of The Literature ◽  
Massive Lymphadenopathy ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.

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