| Autor(en) |
Titel |
Quelle |
Links |
| Hadfield PJ, Birchall MA, Albert DM |
Otitis externa in Langerhans' cell histiocytosis--the successful use of topical nitrogen mustard. |
International journal of pediatric otorhinolaryngology 1994, 30: 143 |
|
| Otitis externa is a common symptom of Langerhans' cell histiocytosis, and occurs in half of all children with the disease. Previous treatment regima have been either unsuccessful, or associated with undesirable side-effects. This study describes the successful use of topical 20% nitrogen mustard ear drops in the management of five children who had Langerhans' cell histiocytosis with otitis externa. All of them had previously been resistant to other forms of treatment. The children were aged between 6 months and 6 years. The otitis externa fee period to date is up to 5 years. Topical nitrogen mustard ear drops are rapidly effective, can be administered at home, have not caused any adverse effects, and are of long-term benefit. |
| Emile JF, Charlotte F, Amoura Z, Haroche J |
BRAF mutations in Erdheim-Chester disease. |
Journal of clinical oncology : official journal of the American Society of Clinical Oncology 2013 Jan 20; 31: 398 |
|
| Haroche J, Cohen-Aubart F, Emile JF, Arnaud L, Maksud P, Charlotte F, Cluzel P, Drier A, Hervier B, Benameur N, Besnard S, Donadieu J, Amoura Z |
Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. |
Blood 2013 Feb 28; 121: 1495 |
|
| Histiocytoses are rare disorders of unknown origin with highly heterogeneous prognosis. BRAFV600E gain-of-function mutations have been observed in 57% of cases of Langerhans cell histiocytosis (LCH) and 54% of cases of Erdheim-Chester disease (ECD), but not in other types of histiocytoses. Targeted therapy with an inhibitor of mutated BRAF (vemurafenib) improves survival of patients with melanoma. Here, we report vemurafenib treatment of 3 patients with multisystemic and refractory ECD carrying the BRAFV600E mutation; 2 also had skin or lymph node LCH involvement. The patients were assessed clinically, biologically (CRP values), histologically (skin biopsy), and morphologically (positron emission tomography [PET], computed tomography and magnetic resonance imaging). For all patients, vemurafenib treatment led to substantial and rapid clinical and biologic improvement, and the tumor response was confirmed by PET, computed tomography, and/or magnetic resonance imaging 1 month after treatment initiation. For the first patient treated, the PET response increased between months 1 and 4 of treatment. The treatment remained effective after 4 months of follow-up although persistent disease activity was still observed. Treatment with vemurafenib, a newly approved BRAF inhibitor, should be considered for patients with severe and refractory BRAFV600E histiocytoses, particularly when the disease is life-threatening. |
| Haroche J, Arnaud L, Cohen-Aubart F, Hervier B, Charlotte F, Emile JF, Amoura Z |
Erdheim-Chester disease. |
Rheumatic diseases clinics of North America 2013, 39: 299 |
|
| Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis. Diagnosis of ECD is based on the identification in tissue biopsy of histiocytes, which are typically foamy and immunostain for CD68+ CD1a-. Central nervous system involvement is a major prognostic factor in ECD. Interferon alpha may be the best first-line therapy and significantly improves survival of ECD. The BRAFV600E mutation is found in more than 50% of cases. Vemurafenib has been used for a small number of patients harbouring this mutation; inhibition of BRAF activation by vemurafenib was highly beneficial in these cases of severe multisystemic and refractory ECD. |
| Haroche J, Arnaud L, Cohen-Aubart F, Hervier B, Charlotte F, Emile JF, Amoura Z |
Erdheim-Chester disease. |
Current rheumatology reports 2014, 16: 412 |
|
| Erdheim-Chester disease (ECD) is a rare (approximately 500 known cases worldwide), non-inherited, non-Langerhans form of histiocytosis of unknown origin, first described in 1930. It is characterized by xanthomatous or xanthogranulomatous infiltration of tissues by foamy histiocytes, |
| Haupt R, Nanduri V, Calevo MG, Bernstrand C, Braier JL, Broadbent V, Rey G, McClain KL, Janka-Schaub G, Egeler RM |
Permanent consequences in Langerhans cell histiocytosis patients: a pilot study from the Histiocyte Society-Late Effects Study Group. |
Pediatric blood & cancer 2004, 42: 438 |
|
| Permanent consequences (PC) are often described among subjects with Langerhans cell histiocytosis (LCH) but data on the real incidence are scarce. Within the Histiocyte Society (HS), and in order to design a definitive late effects study, a retrospective survey was organized to describe the prevalence of PC among long-term survivors of LCH. |
| Haupt R, Nanduri VR, Calevo MG, Egeler RM |
Late effects of Langerhans cell Histiocytosis and the association of LCH with malignancy. |
Cambridge University Press: Histiocytic Disorders in Children and Adults (ed. by Weitzman S, Egeler RM) 2005 |
|
| Haupt R, Minkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, Egeler RM, Janka G, Micic D, Rodriguez-Galindo C, Van Gool S, Visser J, Weitzman S, Donadieu J, Euro Histio Network |
Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. |
Pediatric blood & cancer 2013, 60: 175 |
|
| These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was available in the literature up to 2012. Data and publications have been ranked according to evidence based medicine and when there was a lack of published data, consensus between experts was sought. Guidelines for diagnosis, initial clinical work-up, and treatment and long-term follow-up of LCH patients are presented. |
| Herbrüggen H, Lakatos K, Gadner H, Minkov M |
Isolated cutaneous Langerhans cell histiocytosis in a premature baby: what is the optimal approach? |
Pediatric blood & cancer 2013, 60: 163 |
|
| Heyd R, Strassmann G, Donnerstag F, Martin T, Zamboglou N |
[Radiotherapy in Langerhans-cell histiocytosis. 2 case reports and review of the literature]. |
Rontgenpraxis; Zeitschrift fur radiologische Technik 2000, 53: 51 |
|
| The use of radiotherapy in the treatment of Langerhans' cell histiocytosis was first reported in the literature in 1930 and has been proven as effective in numerous studies. We present the results of two female adults with eosinophilic granuloma of bone who underwent conventionally fractionated radiation therapy with total doses of 7 x 1.8 Gy and 7 x 2.0 Gy in four different sites. After observation periods raging from three months to six years local control of the disease was achieved in all treated locations. A review of 18 previously published studies include a total of 310 sites of eosinophilic granuloma of bone in 216 patients. It was demonstrated in 13 studies that the patients had complete relief of symptoms. An average of 94.3% had local control of the symptoms. Furthermore, in 12 studies for a total of 344 cases with involvement of other organs local control was reported in an average of 64.8% (range: 14.3-100%). Based on our own observations and on the literature review we conclude that low dose radiation therapy plays an important role in the management of localised Langerhans' cell histiocytosis. In order to minimise the risk of radiation induced neoplasms an accurate and precise radiation technique is required. |
| Hoeger PH, Nanduri VR, Harper JI, Atherton DA, Pritchard J |
Long term follow up of topical mustine treatment for cutaneous langerhans cell histiocytosis. |
Archives of disease in childhood 2000, 82: 483 |
|
| Skin lesions in Langerhans cell histiocytosis (LCH) are often painful and difficult to treat. Topical application of nitrogen mustard (0.02% mechlorethamine hydrochloride, mustine), an alkylating cytostatic agent, has been shown to be effective. There is, however, concern about potentially harmful long term side effects. |
| 11 items found |