| Autor(en) |
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| Dauriat G, Mal H, Thabut G, Mornex JF, Bertocchi M, Tronc F, Leroy-Ladurie F, Dartevelle P, Reynaud-Gaubert M, Thomas P, Pison C, Blin D, Stern M, Bonnette P, Dromer C, Velly JF, Brugière O, Lesèche G, Fournier M |
Lung transplantation for pulmonary langerhans' cell histiocytosis: a multicenter analysis. |
Transplantation 2006 Mar 15; 81: 746 |
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| Lung transplantation (LT) may represent a therapeutic option in case of advanced pulmonary Langerhans' cell histiocytosis (PLCH). Little is known however about the characteristics of the patients considered for LT or its results. |
| Derenzini E, Fina MP, Stefoni V, Pellegrini C, Venturini F, Broccoli A, Gandolfi L, Pileri S, Fanti S, Lopci E, Castellucci P, Agostinelli C, Baccarani M, Zinzani PL |
MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: experience on seven patients. |
Annals of oncology : official journal of the European Society for Medical Oncology / ESMO 2010, 21: 1173 |
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| Adult Langerhans cell histiocytosis (LCH) is a rare disease. The combination of vinblastine and prednisone, given in a 6-month course, is the standard of care but prospective randomized trials are lacking. |
| DiMaggio LA, Lippes HA, Lee RV |
Histiocytosis X and pregnancy. |
Obstetrics and gynecology 1995, 85(5 Pt 2): 806 |
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| Histiocytosis X, a clinically heterogeneous infiltrating disorder, is rarely associated with pregnancy. Diabetes insipidus is a common manifestation of histiocytosis X. |
| Diamond EL, Dagna L, Hyman DM, Cavalli G, Janku F, Estrada-Veras J, Ferrarini M, Abdel-Wahab O, Heaney ML, Scheel PJ, Feeley NK, Ferrero E, McClain KL, Vaglio A, Colby T, Arnaud L, Haroche J |
Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. |
Blood 2014 May 21; |
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| Erdheim-Chester Disease (ECD) is a rare non-Langerhans histiocytosis. Recent findings suggest that ECD is a clonal disorder, marked by recurrent BRAFV600E mutations in more than 50% of patients, in which chronic uncontrolled inflammation is an important mediator of disease pathogenesis. Although approximately 500-550 cases have been described in the literature to date, increased physician awareness has driven a dramatic increase in ECD diagnoses over the last decade. ECD frequently involves multiple organ systems and has historically lacked effective therapies. Given the protean clinical manifestations and the lack of a consensus-derived approach for the management of ECD, we provide here the first multidisciplinary consensus guidelines for the clinical management of ECD. These recommendations were outlined at the First International Medical Symposium for ECD, comprised of a comprehensive group of international academicians with expertise in the pathophysiology and therapy of ECD. Detailed recommendations on the initial clinical, laboratory, and radiographic assessment of ECD patients are presented in addition to treatment recommendations based on critical appraisal of the literature and clinical experience. These formalized consensus descriptions will hopefully facilitate ongoing and future research efforts in this disorder. |
| Dimentberg RA, Brown KL |
Diagnostic evaluation of patients with histiocytosis X. |
Journal of pediatric orthopedics 1990 Nov-Dec; 10: 733 |
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| The natural history of histiocytosis X was reviewed in 52 patients to identify the characteristics of patients with localized versus disseminated disease. Two categories of patients were identified. Older patients with solitary or multiple bone lesions constituted a low-risk group. This group had a high rate of development of new bone lesions (55% were asymptomatic) but an excellent prognosis. Patients with soft tissue involvement constituted a group at high risk for disease progression and organ dysfunction. These patients required a more extensive diagnostic workup and systemic treatment. A diagnostic algorithm is proposed to identify these two groups. |
| Dogan AS, Conway JJ, Miller JH, Grier D, Bhattathiry MM, Mitchell CS |
Detection of bone lesions in Langerhans cell histiocytosis: complementary roles of scintigraphy and conventional radiography. |
Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology 1996, 18: 51 |
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| PURPOSE: This research was undertaken to determine the relative sensitivity of scintigraphic and radiographic bone survey examinations in detecting bone lesions in various regions of the skeleton in patients with a histopathologic diagnosis of Langerhans cell histiocytosis (LCH).
PATIENTS AND METHODS: Radionuclide skeletal scintigraphy (RNSS) and x-ray skeletal surveys (XRSS) of 42 children with a histopathologic diagnosis of LCH were evaluated retrospectively.
RESULTS: Of the 191 lesions detected in 42 patients, 36 (19%) were missed on RNSS and 55 (29%) were missed on XRSS. Most of the missed lesions on RNSS were in the skull (26 of 36). XRSS also missed 13 skull lesions seen on RNSS. Most of the lesions missed on XRSS were in the ribs (20 of 30 rib lesions), whereas RNSS identified 29 of them. XRSS also missed 13 of 38 lesions in the spine and pelvis, while RNSS demonstrated all of them. Both modalities showed comparable sensitivity in the extremities.
CONCLUSION: RNSS has a greater value in detecting sites of bone involvement with LCH than reported previously. RNSS is more sensitive than XRSS in detecting histiocytic lesions in the ribs, spine and pelvis and less sensitive in identifying lesions in the skull. |
| Donadieu J, Piguet C, Bernard F, Barkaoui M, Ouache M, Bertrand Y, Ibrahim H, Emile JF, Hermine O, Tazi A, Genereau T, Thomas C |
A new clinical score for disease activity in Langerhans cell histiocytosis. |
Pediatric blood & cancer 2004, 43: 770 |
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| To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH). |
| Donadieu J, Rolon MA, Thomas C, Brugieres L, Plantaz D, Emile JF, Frappaz D, David M, Brauner R, Genereau T, Debray D, Cabrol S, Barthez MA, Hoang-Xuan K, Polak M, French LCH Study Group |
Endocrine involvement in pediatric-onset Langerhans' cell histiocytosis: a population-based study. |
The Journal of pediatrics 2004, 144: 344 |
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| To document the frequency and outcome of endocrine involvement in pediatric-onset Langerhans' cell histiocytosis (LCH), and the association with other types of organ involvement. |
| Donadieu J, Rolon MA, Pion I, Thomas C, Doz F, Barkaoui M, Robert A, Deville A, Mazingue F, David M, Brauner R, Cabrol S, Garel C, Polak M, French LCH Study Group |
Incidence of growth hormone deficiency in pediatric-onset Langerhans cell histiocytosis: efficacy and safety of growth hormone treatment. |
The Journal of clinical endocrinology and metabolism 2004, 89: 604 |
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| We retrospectively studied 61 patients with GH deficiency (GHD), identified among 589 patients with Langerhans cell histiocytosis (LCH) enrolled in a nationwide survey between 1993 and 2001. Overall, 141 patients in the survey developed diabetes insipidus. The median follow-up of the 61 patients with GHD was 12 yr. The 5- and 10-yr risks of GHD among patients with diabetes insipidus were 34.7 +/- 4.5% and 53.7 +/- 5.2%, respectively. Growth velocity decreased soon after LCH diagnosis in patients who developed GHD, and anterior pituitary height, estimated by magnetic resonance imaging, was significantly reduced relative to patients who remained free of GHD. GH replacement therapy was administered to 47 of the 61 patients with GHD. Among GH-treated patients, median final height (-0.8 SD) was significantly greater than median height at GHD diagnosis (-1.6 SD) but remained below midparental (target) height. Among patients with pituitary involvement, the number of LCH disease episodes appeared not significantly influenced by GHD or GH administration, suggesting an absence of deleterious effect of GH therapy on LCH disease activity. |
| Ducassou S, Seyrig F, Thomas C, Lambilliotte A, Marec-Berard P, Berger C, Plat G, Brugiere L, Ouache M, Barkaoui M, Armari-Alla C, Lutz P, Leverger G, Rialland X, Mansuy L, Pacquement H, Jeziorski E, Gandemer V, Chalard F, Chateil JF, Tazi A, Emile JF, Donadieu J, Investigators of the French LCH Study Group |
Thymus and mediastinal node involvement in childhood Langerhans cell histiocytosis: long-term follow-up from the French national cohort. |
Pediatric blood & cancer 2013, 60: 1759 |
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| Mediastinal involvement (MI) in Langerhans cell histiocytosis (LCH) has been rarely reported. Here, we describe the clinical, radiological, and biological presentation, and the outcome of childhood LCH with MI. |
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