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 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 of the Thyroid Leading to the Diagnosis of a Disseminated Form

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Ibtissem Ben Nacef ◽  
Sabrine Mekni ◽  
Chedi Mhedhebi ◽  
Ines Riahi ◽  
Imen Rojbi ◽  
...  
Keyword(s):  
Thyroid Gland ◽  
Thyroid Nodule ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Histological Study ◽  
S100 Protein ◽  
Langerhans Cell ◽  
Multisystemic Disease ◽  
Right Lobe ◽  
The Right

Langerhans cell histiocytosis (LCH) is a rare sporadic proliferative disorder of Langerhans cells. LCH rarely involves the thyroid gland. We report herein a case of a disseminated chronic form of LCH with a diagnosis established by histological examination of the thyroid gland. It is about a 37-year-old female who underwent total thyroidectomy for a thyroid nodule of the right lobe. Histological study showed a granulomatous thyroiditis, and the immunohistochemistry study revealed a strong positivity of histiocytes for the CD1 antigen and for the S100 protein. The incidence of LCH involving the thyroid gland, either as an isolated lesion or as a part of multisystemic disease, is extremely rare.

scholarly journals Recurrent BRAF mutations in Langerhans cell histiocytosis

Blood ◽  
2010 ◽  
Vol 116 (11) ◽  
pp. 1919-1923 ◽  
Author(s):  
Gayane Badalian-Very ◽  
Jo-Anne Vergilio ◽  
Barbara A. Degar ◽  
Laura E. MacConaill ◽  
Barbara Brandner ◽  
...  
Keyword(s):  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Braf V600e ◽  
Neoplastic Disease ◽  
Braf Mutations ◽  
Multiple Organs ◽  
Mitogen Activated Protein ◽  
Formalin Fixed Paraffin Embedded

Abstract Langerhans cell histiocytosis (LCH) has a broad spectrum of clinical behaviors; some cases are self-limited, whereas others involve multiple organs and cause significant mortality. Although Langerhans cells in LCH are clonal, their benign morphology and their lack (to date) of reported recurrent genomic abnormalities have suggested that LCH may not be a neoplasm. Here, using 2 orthogonal technologies for detecting cancer-associated mutations in formalin-fixed, paraffin-embedded material, we identified the oncogenic BRAF V600E mutation in 35 of 61 archived specimens (57%). TP53 and MET mutations were also observed in one sample each. BRAF V600E tended to appear in younger patients but was not associated with disease site or stage. Langerhans cells stained for phospho-mitogen–activated protein kinase kinase (phospho-MEK) and phospho-extracellular signal-regulated kinase (phospho-ERK) regardless of mutation status. High prevalence, recurrent BRAF mutations in LCH indicate that it is a neoplastic disease that may respond to RAF pathway inhibitors.

scholarly journals Langerhans cell histiocytosis of the orbit and frontal sinus of the adult woman: A first case report in Poland

2019 ◽  
Vol 10 ◽  
pp. 234
Author(s):  
Kacper Kostyra ◽  
Bogusław Kostkiewicz
Keyword(s):  
Case Report ◽  
Frontal Sinus ◽  
Langerhans Cell Histiocytosis ◽  
Histopathological Examination ◽  
Langerhans Cell ◽  
Radical Excision ◽  
Multisystem Disease ◽  
Upper Eyelid ◽  
First Case ◽  
The Right

Background: Langerhans cell histiocytosis (LCH) is a term describing a clonal proliferation of pathologic Langerhans cells (histiocytes), which may manifest as unisystem (unifocal or multifocal) or multisystem disease. LCH is a rare cause of the orbital tumor with the predilection to its lateral wall which is particularly common in children. Case Description: We report an unusual case of a 33-year-old woman, 6 months after childbirth, who presented with the edema of the right orbit and upper eyelid with headaches. On physical examination, the patient had a right superior and lateral swelling of the eyelid and the orbit and right enophthalmos, without blurred vision. Magnetic resonance imaging showed well-defined, expansile, intensely homogeneously enhancing mass lesion in the right superolateral orbital rim with the destruction of the upper wall of the orbit, growing into the frontal sinus and frontal part of the cranium with the bold of the dura mater in this region. Radical excision of the tumor was achieved through a right fronto-temporo-orbito-zygomatic craniotomy. Histopathological examination had confirmed the diagnosis of the LCH. The patient was discharged home with a modified Rankin Scale score of 0. Conclusion: The main purpose of this case report is that LCH should be considered as one of the possible causes of quickly appearing tumor of the orbit in adults.

scholarly journals Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report

2013 ◽  
Vol 70 (12) ◽  
pp. 1159-1161
Author(s):  
Aleksandra Lovrenski ◽  
Mirna Djuric ◽  
Istvan Klem ◽  
Zivka Eri ◽  
Milana Panjkovic ◽  
...  
Keyword(s):  
Case Report ◽  
Lung Adenocarcinoma ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Incidental Finding ◽  
Lung Parenchyma ◽  
Langerhans Cell ◽  
Heavy Smoker ◽  
Unknown Etiology ◽  
The Right

Introduction. Langerhans cell histiocytosis (LCH) is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal lesion which penetrated into the right ventricle resulting in internal and external hematocephalus. Histologically and immunohistohemically, the diagnosis of primary lung adenocarcinoma with brain metastasis was made (tumor cells showed positivity for CK7 and TTF-1 which confirmed the diagnosis). In the lung parenchyma around the tumor, as well as in brain tissue around the metastatic adenocarcinoma histiocytic lesions were found. Light microscopic examination of the other organs also showed histiocytic lesions involving the pituitary gland, hypothalamus, spleen and mediastinal lymph nodes. Immunohistochemical studies revealed CD68, S-100 and CD1a immunoreactivity within the histiocytes upon which the diagnosis of Langerhans' cells histiocytosis was made. Conclusion. The multisystem form of LCH with extensive organ involvement was an incidental finding, while metastatic lung adenocarcinoma to the brain that led to hematocephalus was the cause of death.

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.

scholarly journals Adult-Onset Langerhans Cell Histiocytosis And The Role of Vinblastine In The Treatment -Ankara Oncology Hospital Experience

2020 ◽  
Vol 53 (3) ◽  
pp. 492-499
Author(s):  
Ayşegül Tetik ◽  
Bahar Uncu Ulu ◽  
Mehmet Bakırtaş ◽  
Tuğçe Nur Yiğenoğlu ◽  
Jale Yıldız ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Adult Onset ◽  
Hospital Experience
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