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

Histiocytosis X is still of unknown origin. Its clinical patterns are various and it is above all a bone disease of children, mostly boys. It is a tumoral condition basically benign but with a strong tendency toward dissemination and destruction. Its natural history is unpredictable. The authors have reviewed 37 cases after a 44 months mean follow-up for bringing up a better understanding of the role of orthopedic surgery. The diagnosis was always based on biopsies looking for at least two basic signs out of four possible ones. Scintigraphy was used to detect other locations. MRI was used to evaluate the effect of treatment. In single eosinophilic granuloma, curettage was associated with biopsy. Local injections of corticoids may help in spinal locations. In disseminated forms, surgical treatment should be little aggressive, limited to a biopsy of the most superficial lesion. These forms should be treated by radiotherapy corticotherapy and chemotherapy, as well as the aggressive lesions, the extensive ones, the threatening ones and those which are not accessible.
 ...
PMID:[Localizations of histiocytosis X in bone]. 141 Jul 17

The records of 70 patients presenting to this hospital since 1961 with histiocytosis X confirmed by biopsy examination have been reviewed. The patients were subdivided into three groups: group A, those under 2 years of age at diagnosis; group B, those between 2 and 5 years; and group C, those over 5 years. All eight patients who died (11% overall mortality) were under 2 years of age at diagnosis. Involvement of lung, liver, and bone marrow were confirmed as poor prognostic features. The presence of bone disease and absence of skin rash were identified as favourable features.
 ...
PMID:Favourable prognostic features in histiocytosis X: bone involvement and absence of skin disease. 349 68

Treatment of Langerhans cell histiocytosis (LCH) remains problematic. To test the hypothesis that rapid initiation and long-term continuation of chemotherapy can improve survival and reduce recurrence and late consequences of disseminated LCH, we have completed a prospective clinical trial (DAL HX-83). One hundred six newly diagnosed patients were stratified into three risk groups (A: multifocal bone disease [n = 28]; B: soft tissue involvement without organ dysfunction [n = 57]; C: organ dysfunction [n = 21]). All patients received an identical initial 6-week treatment (etoposide [VP-16], prednisone, and vinblastine), and continuation treatment for 1 year, slightly adapted according to stratification at diagnosis. It included oral 6-mercaptopurine and eight pulses of vinblastine and prednisone for all patients, plus VP-16 in group B and VP-16 and methotrexate in group C. Eighty-nine percent and 91% of patients in groups A and B and 67% of the most severely affected group C, achieved complete resolution of disease. The speed of resolution was rapid (median 4 months) and independent of disease severity. The frequency of recurrence after initial resolution was low (12%, 23%, and 42% in groups A, B and C); overall fully 77% of patients have remained free of recurrence. Permanent consequences developed after diagnosis in 20% of the patients. Diabetes insipidus after initiation of treatment occurred in only 10% of patients. Mortality (9%) was limited to patients of groups B (two patients) and C (eight patients). Finally, among the 106 patients treated by DAL HX-83 none have developed a malignancy (median follow-up 6 years, 9 months). The shorter duration of active disease, low rate of recurrence and permanent consequences, and improved survival among patients with poor prognosis support the strategy of rapid initiation of a predefined prolonged treatment upon the diagnosis of disseminated LCH.
 ...
PMID:Treatment strategy for disseminated Langerhans cell histiocytosis. DAL HX-83 Study Group. 820 45

Langerhans cell histiocytosis (LCH) is a clonal neoplastic disorder that results in a spectrum of clinical manifestations. Known to be associated with a variety of malignant diseases, LCH may precede, coincide with, or develop after the diagnosis of cancer. A child with a malignant germ cell tumor of the brain who subsequently experienced LCH is reported. The 8-year-old boy was treated for an immature teratoma of the posterior fossa with gross total resection and craniospinal irradiation preceding bleomycin, etoposide, and vinblastine chemotherapy for four cycles. Seven months after completion of therapy, he experienced multifocal bone disease with LCH.
 ...
PMID:Langerhans cell histiocytosis after therapy for a malignant germ cell tumor of the central nervous system. 1095 8

Little is known about the cause, nature, treatment and prognosis of pulmonary Langerhans' cell histiocytosis (LCH) in adults. We report the case of a 44-year-old female non-smoker suffering from pulmonary histiocytosis who after a 7-year remission period relapsed with both lung and bone disease. Using a combination of corticosteroids, methotrexate and bone irradiation treatment, the patient achieved total disease remission. The patient was a non-smoking female who has had long-term and swift remission of the disease on two occasions.
 ...
PMID:Progressive form of pulmonary Langenharns' cell histiocytosis in a female adult non-smoker. 1470 54

The predominant clinical and radiological features of Langerhans' cell histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation; nearly all anatomical sites may be affected and in very various combinations. The following article is based on a multicentre review of 31 children with extra-osseous LCH. The objective is to summarise the diverse possibilities of organ involvement. The radiological manifestations using different imaging modalities are rarely pathognomonic on their own. Nevertheless, familiarity with the imaging findings, especially in children with systemic disease, may be essential for early diagnosis.
 ...
PMID:Extra-osseous involvement of Langerhans' cell histiocytosis in children. 1474 Feb 1

We report a case of a girl with Langerhans cell histiocytosis (LCH) of multifocal bone disease, who developed recurrent bacterial meningitis and unilateral sensorineural hearing loss during the relapsing course of the disease. Mondini dysplasia, a congenital inner ear anomaly, was suspected by high resolution computed tomographic scan and the dysplasia with cerebrospinal fluid leakage was confirmed by surgery in the ipsilateral ear showing hearing loss. Although rare, congenital inner ear anomalies such as Mondini dysplasia should be kept in mind in pediatric patients with hearing impairment and/or recurrent bacterial meningitis during chemotherapy for various types of neoplasms including LCH.
 ...
PMID:Mondini dysplasia and recurrent bacterial meningitis in a girl with relapsing Langerhans cell histiocytosis. 1517 Aug 97

The predominant clinical and radiological features of Langerhans Cell Histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation. In the present study, LCH was presumptively diagnosed on Ultrasound guided Fine needle aspiration cytology (FNAC) of the mediastinal lymph node in a 18 month-old child. The diagnosis was confirmed by histological examination of the biopsy material.S-100 protein localization in the LCH cells is often positive on immunohistochemistry.
 ...
PMID:Langerhans cell histiocytosis of mediastinal node. 1837 3

The aim of this study was to assess the outcome of treatment with 2-chlorodeoxyadenosine (2-CdA) in pediatric patients with Langerhans cell histiocytosis (LCH) in Japan. We retrospectively identified 17 pediatric LCH patients treated with 2-CdA. All patients were refractory or reactivated cases who had been initially treated according to the JLSG-02 protocol of the Japan LCH study group. At initiation of 2-CdA therapy, 4 patients had primary refractory multisystem (MS) disease with risk organ (RO) involvement (MS+), 9 patients had reactivated MS disease [5 MS+ and 4 without RO involvement (MS-)], and the remaining 4 patients had refractory/reactivated multifocal bone disease (MFB). Treatment with 2-CdA (4-9 mg/m(2)/day) was administered on 2-5 consecutive days and repeated every 3-4 weeks for a period that ranged from 2 to 12 months. Four primary refractory patients were treated with 2-CdA combined with high dose of cytarabine. In MS+ patients, response to treatment was observed in 5 of the 9 patients. In MS-/MFB patients, 5 of the 8 patients showed response to treatment. In the patients who were primary refractory or had reactivation during initial chemotherapy, 4 of 10 patients showed good response. On the other hand, in the patients having reactivation while off therapy, 6 of 7 patients showed good response. These findings suggest that 2-CdA is effective for reactivated LCH while off therapy.
 ...
PMID:Outcome of pediatric patients with Langerhans cell histiocytosis treated with 2 chlorodeoxyadenosine: a nationwide survey in Japan. 2036 Dec 77

Our aim is to provide review of available studies on Langerhans cell histiocytosis (LCH) and discuss treatment for polyostotic monosystem form of disease based on our clinical experience. LCH is an enigmatic disease with insufficiently understood etiology, pathophysiology, and variety of clinical presentations ranging from solitary eosinophilic granuloma to severe multisystem disease. It is marked by formation of granuloma in practically any organ. We present rare case of multifocal bone disease in 36-year old patient without visceral involvement. Treatment protocols for adult LCH patients, especially for uncommon form in our case have not yet been defined. Our therapeutical trial with corticosteroids showed limited success with numerous side-effects. We conclude that LCH treatment can commence only after diagnosis and staging of the disease. Other factors like patient's age, comorbidity, general condition, severity of symptoms and contraindications for therapy modalities should also be considered. In our experience expectative approach has better clinical outcome than immunosuppressive therapy in patients suffering from polyostotic multifocal form LCH with mild symptoms.
 ...
PMID:Monosystem multifocal Langerhans cell histiocytosis (multifocal eosinophilic granulomas of the bone) in a 36-year old patient: case report, therapeutic doubts and review of literature. 2394 Sep 99


1 2 Next >>