Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This study evaluated the outcome of patients with symptomatic bone Langerhans cell histiocytosis (LCH) treated with indomethacin alone, either at diagnosis or after reactivation (after recurrence with previous therapies). We evaluated the nonrandomized use of oral indomethacin (2 mg/kg/d) in patients with symptomatic single-system bone LCH. From 1997 to 2012, 38 sequential patients were treated for a median of 4 months. Criteria of nonactive disease (NAD) after initial treatment (8 wk) were: no pain, no soft tissue involvement, no increase of size, or no new bone lesions. Twenty-two patients were treated at diagnosis: 18 showed NAD after initial treatment (2 patients who had bone reactivations were retreated with indomethacin and remain with NAD). Three patients improved and they are with NAD after treatment with indomethacin, steroids, or radiotherapy. One patient developed progressive bone disease and he is with NAD after treatment with steroids and chemotherapy. Sixteen patients were treated after reactivation, and all were with NAD after initial treatment: 5 reactivated and 4 remain with NAD after retreatment with indomethacin. Toxicity was not significant. We conclude that indomethacin is a well tolerated and active drug in patients with symptomatic bone disease. The results support the concept that chemotherapy may not be necessary for limited bone disease.
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PMID:Symptomatic bone langerhans cell histiocytosis treated at diagnosis or after reactivation with indomethacin alone. 2497 2

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unknown etiopathogenesis. Its clinical presentation is variable and ranges from isolated skin or bone disease to a life-threatening multisystem condition. LCH can occur at any age but is more frequent in the pediatric population. The diagnosis depends on clinical, histopathological and radiographic examination and should be confirmed by immunohistochemical study with CD1a, S100 protein and langerin, three markers used widely for identifying Langerhans cells. Herein, we report an adolescent with acute myeloid leukemia (AML-M2) who was treated just with surgical management alone for LCH. As far as we know, this is the first case that the LCH patient without chemotherapy evolved into AML and was successfully cured. Cooperative studies of large numbers of LCH patients are needed to evaluate a possible association between LCH and acute leukemia, and to identify common risk factors or predisposing agents if such be present. The previously reported cases of LCH concomitant with other hematological disorders are also summarized and described compared with the present case.
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PMID:Successful treatment of a case of acute myeloid leukemia following Langerhans cell histiocytosis in an adolescent: a case report and review of the literature. 2593 77

Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence.
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PMID:Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule. 2658 39

This article presents the use of bone cytology for diagnosis of bone tumors. It discusses critical factors and considerations of fine-needle aspiration and bone cytology and presents diagnostic options and differential diagnosis for benign and malignant bone lesions. Osteomyelitis, chrondroblastoma, Langerhans cell histiocytosis, chondromyxoid fibromas, enchondromas, giant cell tumor of bone, osteosarcoma, chondrosarcoma and variants, Ewing sarcoma, chordoma, plasmacytoma, multiple myeloma, lymphoma, and metastatic bone disease are presented.
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PMID:Bone Cytology: A Realistic Approach for Clinical Use. 2683 16

This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.
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PMID:Pediatric langerhans cell histiocytosis: single center experience over a 17-year period. 2827 5

Erdheim-Chester disease (ECD) is a rare type of non-Langerhans cell histiocytosis, with only 550 cases reported worldwide. ECD is characterized by diffuse histiocytic infiltration of multiorgans. The age of presentation of this disease is typically between 40 and 70 years. Bone disease is the most common symptom, as unique radiological findings of long bone sclerosis occur in 96% of cases. Furthermore, BRAF V600E mutation is detected in 60% of ECD cases. In this manuscript, we are describing a unique case of ECD; the patient is younger than most reported cases and has no bone pain or any skeletal involvement. This patient has unintentionally lost about 50% of his body mass and is suffering from progressive cerebellar manifestations with radiological evidence of cerebellar atrophy, in contrast to the usual ECD manifestation of cerebellar infiltration. In addition, the patient has cardiac, retroperitoneal, and perinephric involvement, but he retains his sexual drive and fertility. A tissue biopsy from the retroperitoneal mass displayed typical morphological and immunohistochemical features of ECD, and BRAF V600E mutation was detected. He was treated with pegylated interferon alpha, but his disease progressed and the treatment was changed to vemurafenib to which he had an excellent response at 6 weeks.
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PMID:Erdheim-Chester Disease with No Skeletal Bone Involvement and Massive Weight Loss. 2921 86


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