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histiocytosis x and clinical cases

Lynn H Lee, Anjelika Gasilina, Jayeeta Roychoudhury, Jason Clark, Francis X McCormack, Joseph Pressey, Michael S Grimley, Robert Lorsbach, Siraj Ali, Mark Bailey, Philip Stephens, Jeffrey S Ross, Vincent A Miller, Nicolas N Nassar, Ashish R Kumar
Many patients with histiocytic disorders such as Langerhans cell histiocytosis (LCH) or Erdheim-Chester disease (ECD) have treatment-refractory disease or suffer recurrences. Recent findings of gene mutations in histiocytoses have generated options for targeted therapies. We sought to determine the utility of prospective sequencing of select genes to further characterize mutations and identify targeted therapies for patients with histiocytoses. Biopsies of 72 patients with a variety of histiocytoses underwent comprehensive genomic profiling with targeted DNA and RNA sequencing...
February 9, 2017: JCI Insight
Emmanuel Dhiravia Sargunam Azariah, Deepak Chandrasekaran, Ravindran Chinnaswami, Sivaramakrishnan Balasubramaniam, Eswari Jagdish
Histiocytosis X is an idiopathic disease, characterized by a disorder of the reticulo-endothelial system in the human body. Histopathological studies carried out right from the 1800s have seen a significant similarity in the pathologic process of different stages in particular clinical syndromes showing proliferation of mature histiocytes. It was then modified by Lichenstein in 1953 as "Histiocytosis X". The exact aetiology is unknown; hence, the name "Histiocytosis X". The disease classically presents with three syndromes namely Eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian disease...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
Julie Obert, Laetitia Vercellino, Axel Van Der Gucht, Constance de Margerie-Mellon, Emmanuelle Bugnet, Sylvie Chevret, Gwenaël Lorillon, Abdellatif Tazi
PURPOSE: The standard evaluation of multisystem Langerhans cell histiocytosis (LCH) includes a clinical evaluation, laboratory tests and a skeleton/skull X-ray survey, with chest high-resolution computed tomography (HRCT) in the case of pulmonary involvement. Preliminary reports suggest that (18)F-fluorodeoxyglucose positron emission tomography-computed tomography ((18)F-FDG PET-CT) may be useful for evaluating patients with LCH. METHODS: Fourteen consecutive adult patients with multisystem LCH were included in this retrospective study, and were evaluated using standard procedures and (18)F-FDG PET-CT...
April 2017: European Journal of Nuclear Medicine and Molecular Imaging
Alex Vargas, Hernán Ramírez, Pablo Ramírez, Camila Foncea, Bernardo Venegas, Paula Astorga
BACKGROUND: Langerhans cell histiocytosis (LCH), previously known as Histiocytosis X, is an infrequent disease that congregates a wide spectrum of clinical presentations with variable systemic involvement. Unification of these diseases under only one category is based on the almost identical histopathologic features of the lesions, but the etiology and proper approach for each presentation remains controversial. The localized alternative of Langerhans cell histiocytosis (LLCH), known as Eosinophilic Granuloma (EG) of bone, is the predominant clinical presentation of LCH...
June 2, 2016: Head & Face Medicine
Emad E Abdellateef, Ayman R Abdelhai, Heba H Gawish, Ghada A Abdulmonaem, Eman H Abdelbary, Ahmed I Ahmed
BACKGROUND: Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis of unknown etiology with multi-organ involvement. CASE REPORT: A 19-year-old woman presented with orthopnea, severe fatigue, bilateral exophthalmos, and gradual loss of vision. She had anemia and mild leucocytosis related to chronic illness. Marked left side pleural effusion and massive pericardial effusion with bilateral hydronephrosis were detected by plain X-ray, echocardiography, and computed tomography, respectively...
May 30, 2016: American Journal of Case Reports
Wei-Fang Chang, Yi-Chih Hsu, Yi-Der Wu, Chun-Lang Kuo, Guo-Shu Huang
Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors...
2016: EXCLI journal
Prasanna Karki, Hirofumi Hirano, Hitoshi Yamahata, Shingo Fujio, Hajime Yonezawa, Koji Iida, Manoj Bohara, Tatsuki Oyoship, Ryosuke Hanaya, Kazunori Arita
Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as a unisystem (unifocal or multifocal) or multisystem disease. We describe the clinical and histologic spectrum of LCH of the orbit and skull in our two cases. Both cases had unifocal erosive skull lesions with a history of trauma. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes...
December 2015: Hiroshima Journal of Medical Sciences
Cuihong Lian, Yuan Lu, Siyuan Shen
BACKGROUND: Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children. Immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. OBJECTIVE: Langerhans cell histiocytosis (LCH) is easy to be misdiagnosed because of its various clinic features and laboratory results. This research focused on the clinicopathological, histopathological, immunohistochemical and other features of LCH and aimed to analyze LCH clinical features for improving diagnosis and decreasing misdiagnosis rate...
April 5, 2016: Oncotarget
Salma Bhat, Parvez Nazir, Humaira Bashir, Ruby Reshi, Sheema Sheikh, Rohi Wani
BACKGROUND: Eosinophilic granuloma (EG) of bone refers to a generally benign form of Langerhans cell histiocytosis localized to the bone. Patients may present with a solitary lesion (monostotic) or multiple sites of involvement (polyostotic). MATERIALS AND METHODS: This study was done to evaluate the clinicopathological pattern of 6 cases of EGs of the skull diagnosed at a tertiary care hospital. All patients of EG were included with the help of medical records over a 5-year period that is, November 2009 to November 2014...
July 2015: Indian Journal of Medical and Paediatric Oncology
Jie Xu, Chun-Hua Liu, Yan-Si Wang, Chang-Xian Chen
Few studies have used fine-needle aspiration cytology for the purpose of isolated skeletal Rosai-Dorfman diseases (RDDs) diagnosis.Herein, we described an extremely rare case of a 56-year-old woman who presented to our hospital with an insidious onset of pain in the right proximal tibia.The provisional cytologic diagnosis of RDDs was confirmed by a computer tomography-guided core needle biopsy of the lesion. Subsequently, curettage and autogenous iliac crest bone graft were performed successfully. At the 4th year of follow-up her clinical symptoms disappeared, and there was no clinical evidence of lesion recurrence...
November 2015: Medicine (Baltimore)
Christopher Lo, Payal Patel, Norman C Charles
The authors describe a 23-year-old man with unilateral upper eyelid swelling that evolved into a multinodular lesion with central necrosis, mimicking a neoplasm. Biopsy showed a lympho-histiocytic, eosinophil-rich proliferation with positivity for Langerhans cell markers CD1a and S-100 and histiocytic marker CD68. A literature review disclosed 11 documented cases of Langerhans cell histiocytosis of the eyelid with variable clinical presentations. This rare eyelid lesion, nearly always solitary, has no clinically distinctive characteristics and requires biopsy for diagnosis...
September 10, 2015: Ophthalmic Plastic and Reconstructive Surgery
J M Blumberg, A Malhotra, X Wu, R K Virk, J F Kveton, E M Michaelides
INTRODUCTION: This study is conducted to demonstrate that destructive lesions of the otic capsule by Langerhans cell histiocytosis (LCH) causing both radiographic and audiologic findings can be completely reversed with adequate treatment. Retrospective case review and analysis of clinical and imaging data were obtained as part of the diagnosis and treatment of patients with LCH of the temporal bone. METHODS: With Institutional Review Board (IRB) approval, cases of LCH involving the temporal bone were searched for within the institutional databases...
September 4, 2015: Clinical Neuroradiology
Mohammed Joudi Aboud, Manal Mohammed Kadhim
Langerhans cell histiocytosis (LCH) as a term encompasses eosinophilic granuloma and two clinical syndromes: Letterer-Siwe disease and Hand-Schüller-Christian. All these syndromes seem to represent similar processes in which the proliferating cells have the structural and functional features of Langerhans cells. In reviewing the literature found that this disease does indeed occur in some families more often than would be expected. We present two boys, siblings with LCH in a single family with two different entities (There were no other siblings) and this paper is presented to emphasize the very rare familial occurrence of EG and the other one with Letterer-Siwe disease in our single family...
2015: SpringerPlus
Domenico Albano, Giovanni Bosio, Francesco Bertagna
Rosai-Dorfman disease (RDD) or sinus histiocytosis is a rare histiocytic disorder characterized by massive lymphadenopathy and with extranodal involvement in 25% to 43% of cases. The clinical course of RDD is unpredictable with episodes of exacerbation and remissions that can last many years, and treatment strategies can be different according to organ involvement. We report a case of a 42-year-old woman with extranodal disease followed for almost 10 years from the diagnosis who underwent seven (18)F-FDG PET/CT...
August 2015: Clinical Nuclear Medicine
Hitoshi Dejima, Shigeki Morita, Yusuke Takahashi, Noriyuki Matsutani, Hisae Iinuma, Fukuo Kondo, Masafumi Kawamura
In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy...
2015: International Journal of Clinical and Experimental Pathology
Cristina Rodríguez Torres, Gema Riazuelo Fantova, Irene Escartín Martínez, Juan José Castillo Escudero, Celia del Agua Arias
We describe two new cases of Rosai-Dorfman disease with intrascrotal involvement. Also known as sinus histiocytosis with massive lymphadenopathy, this is a rare entity, usually presenting with massive bilateral cervical lymphadenopathy. It is associated with extranodal involvement in up to 40% of cases, commonly in the face and neck area. Isolated intrascrotal involvement with no lymphadenopathies is extremely rare, and its definitive diagnosis is histopathological. Although they are the most frequent type, not all intrascrotal lesions are neoplastic; therefore, it is important to know other possible etiologies and their radiogical features that might help in the diagnosis...
August 2015: International Journal of Urology: Official Journal of the Japanese Urological Association
Vruturaj V Shevale, K Ekta, T Snehal, M Geetanjal
Langerhans Cell Histiocytosis (LCH) is a disease process characterized by accumulation and infiltration of cells, showing ultrastructural and immunohistochemical similarities to Langerhans' cell, in the affected tissues. It exhibits extreme clinical heterogeneity. LCH was historically divided into 3 clinical entities based on extent of tissue involvement and severity of presentation. These 3 entities were eosinophilic granuloma, Hand-Schuler-Christian disease, Letterer-Siwe disease. Owing to similarities of their histologic appearance, they were grouped together under the term histiocytosis X...
September 2014: Journal of Oral and Maxillofacial Pathology: JOMFP
Duc Ha, Ruchi Yadav, Peter J Mazzone
Once cystic lung disease is confirmed on computed tomography, one can arrive at the likely diagnosis in most cases by taking a systematic, stepwise approach based on the clinical and radiographic features. Here, we describe the features of cystic lung disease that point to lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, pulmonary angerhans cell histiocytosis, interstitial pneumonia, congenital cystic lung disease, pulmonary infection, and systemic disease.
February 2015: Cleveland Clinic Journal of Medicine
Yadavalli Guruprasad, Dinesh Singh Chauhan
Solitary eosinophilic granuloma (EG) is traditionally included as 1 of the 3 clinical manifestations of the histiocytosis-X group of diseases, which also encompasses Hand-Schuller-Christian disease and Letterer-Siwe disease. EG is the most common lesion in the spectrum of disorders under the classification of Langerhans cell histiocytosis. EG represents the monostotic form of the disease with the head and neck region representing the most common site of initial presentation. We report a rare case of solitary EG involving mandibular condyle in a 49-year-old male patient, which is the first reported case in Indian literature...
March 2015: Journal of Maxillofacial and Oral Surgery
Lalita Fernandes, Rohit Vadala, Anthony Menezes Mesquita, Pradeep Vaideeswar
Adult Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare interstitial lung disease which occurs almost exclusively in smokers. A marked male predominance was initially reported, but recent studies show both men and women are equally affected due to the increasing smoking habits in women. The natural history is variable with 25% of patients having asymptomatic disease while 10-20% progress rapidly to respiratory insufficiency and death. The diagnosis is not easily recognized by clinicians or pathologists...
January 2015: Indian Journal of Tuberculosis
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