Search results for "Langerhans cell histiocytosis"
showing 10 items of 16 documents
Clinical and immunohistochemical evaluation of the vulvar Langerhans cell histiocytosis.
2008
: We present the case of a woman with diabetes insipidus with subsequent genital and multiorgan Langerhans cell histiocytosis (LCH). A monolateral and slightly infiltrated erythematous plaque of the vulva was observed. Hematoxylin and eosin and immunophenotypic studies were performed. The primary antibodies used were monoclonal antibody to S100, CD1a, CD34, HLA-DR, PCNA, CD45Ro, CD40, and langerin. The histology of the infiltrates revealed a granulomatous reaction pattern, with extensive aggregates of histiocyte proliferation. The histiocytes, morphologically characterized by a pale staining of cytoplasm surrounding a grooved reniform nucleus, sometimes contained small distinct nucleoli. L…
Confluens-sinuum-Obstruktion durch Langerhanszell-Histiozytose
1996
Die Langerhanszell-Histiozytose, bis vor wenigen Jahren als Histiozytose X bezeichnet, umfast 3 verschiedene Syndrome, namlich das eosinophile Granulom, die Hand-Schuller-Christian-Krankheit sowie die Abt-Letterer-Siwe-Krankheit, und gehort zu den seltenen Erkrankungen des Kindesalters. Wir berichten uber einen 10 jahrigen Jungen, der mit einer Schwellung am Hinterkopf und beginnender Hirndrucksymptomatik zur stationaren Aufnahme kam. Klinik, konventionelle radiologische Diagnostik und Computertomographie liesen differentialdiagnostisch ein eosinophiles Granulom als wahrscheinlich erscheinen. MRT und MRT-Angiographie zeigten jedoch die akute Bedrohung des Patienten durch das intrakraniale W…
BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups.
2014
BRAF-V600E expression is identified in hematopoietic progenitor and precursor myeloid dendritic cells in patients with high-risk LCH, and enforced expression of BRAF-V600E in CD11c+ cells recapitulates a high-risk LCH-like phenotype in mice.
Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification
2007
Multisystem Langerhans cell histiocytosis (MS-LCH) is associated with high mortality when patients have risk organ involvement (RO+) or are younger than 2 years. In an international randomized trial, LCH-II, we intensified their treatment: arm A consisted of 6 weeks of daily prednisone and weekly vinblastine followed by 18 weeks of daily 6-mercaptopurine with vinblastine/prednisone pulses; etoposide was added in arm B. Considering all 193 randomized risk patients, there were similar outcomes: rapid (6 weeks) response (arm A vs arm B: 63%/71%), 5-year survival probability (74%/79%), disease reactivation frequency (46%/46%), and permanent consequences (43%/37%). However, (1) patients younger …
Response to initial treatment of multisystem langerhans cell histiocytosis: An important prognostic indicator
2002
Background Reliable prediction of prognosis allowing risk-adapted therapy remains a major issue in the management of multisystem Langerhans cell histiocytosis (LCH). In a recent publication of the International LCH Study Group, response to initial therapy appears to be a reliable outcome predictor. The aim of this study is to test this observation in a cohort of patients treated with more intensive initial therapy. Furthermore, we compare the predictive value of response to initial therapy to some other well-established stratification systems. Procedure Response to initial combination chemotherapy (prednisolone, vinblastine, and etoposide) at 6 weeks and its prognostic value was evaluated r…
INTERNATIONAL JOURNAL OF CLINICAL DENTISTRY
2018
Diagnosis and Management of Oral Langerhans Cell Histiocytosis Emanoele Paixão da Silva Silva, Carina Myung Rodenbeck, Monira Samáan Kallas, Cássia Maria Fischer Rubira, and Paulo Sérgio da Silva Santos Mandibular-Infected Buccal Cyst: Unique Radiographic Appearance Gustavo Zanna Ferreira, Cássia Maria Fischer Rubira, Letícia Rodrigues Nery, Alberto Consolaro, Izabel Regina Fischer Rubira-Bullen, and Paulo Sérgio da Silva Santos PRF - The Magical Resort in Surgical Dentistry Archana Mootha, Julie Toby Thomas, Sankari Malaiappan, Sheeja S. Varghese, and N. D. Jayakumar Endodontic Management of a Macrodont: A Rare Tooth Anomaly Rahul Rathi and Priti Saroha Maxillary Canine Root Resorption Con…
A rare case of solitary brain Langerhans cell histiocytosis with intratumoral hemorrhage in a patient affected by Turner syndrome
2016
Background: Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of cells with characteristics similar to bone marrow-derived Langerhans cells. The case of a young woman, affected by Turner syndrome and a solitary intraparenchymal LCH associated with an osteolytic lesion of the overlying skull, is presented. Case Description: The patient, with an insidious history of headache and a growing soft mass in the left frontal region, presented with a sudden generalized tonic-clonic epileptic seizure. Neuroradiological investigations showed an osteolytic lesion of the left frontal bone and an underlying brain lesion associated with recent signs of bleeding. The patie…
Langerhans cell histiocytosis: Current concepts in dentistry and case report
2016
Langerhans cell histiocytosis (LCH), which is a rare granulomatous pediatric disease of unknown etiology, is characterized by the idiopathic proliferation and accumulation of abnormal and clonal Langerhans cells or their marrow precursors, resulting in localized, solitary or multiple destructive lesions. These lesions are most commonly eosinophilic granuloma, which are found in craniofacial bone structures such as the skull and mandible, skin and other organs. In children, the disease has a variable initial presentation, and the clinical course, prognosis and survival are unpredictable. The aims of this report were to present an LCH case in a girl aged 2 years, 8 months and her clinicopatho…
Histiocytosis with mixed cell populations
2016
Langerhans cell histiocytosis (LCH) and juvenile xanthogranuloma (JXG) are thought to originate from a common stem cell precursor, with divergent differentiation under different microenvironmental conditions. We describe an exceptional case of multiple cutaneous lesions in a 10-year-old boy, in which the coexistence of both LCH and JXG cell populations is found in every single lesion. The presence of Birbeck granules and CD207 (langerin) immunostaining in the LCH component would argue against the diagnosis of indeterminate cell histiocytosis (ICH). This unique case gives additional support to the hypothesis of a potentially common histogenesis for LCH and JXG.
Langerhans cell histiocytosis with oral manifestations: a rare and unusual case report
2012
Langerhans cell histiocytosis (LCH), is a rare, proliferative disorder in which the accumulation of pathologic Langerhans cells leads to local tissue infiltration and destruction. We present a case of a 32 years old, completely edentulous female patient who presented with erythema of hard palate, maxillary alveolar mucosa and mucosa over the distobuccal part of mandibular alveolar ridge with foci of ulcerations. Histopathologic features were suggestive of LCH which was confirmed by immunohistochemistry which was CD1a positive, confirmatory for LCH. Bone scan revealed multiple bone involvement. At this stage, disease had already progressed to multisystem involvement with endocrinal abnormali…