Treatment Of Langerhans Cell Histiocytosis Research Articles

Acute promyelocytic leukemia with t(15; 17) abnormality after chemotherapy containing etoposide for langerhans' cell histiocytosis

CancerVolume 74, Issue 5 p. 1650-1652 CorrespondanceFree Access Acute promyelocytic leukemia with t(15; 17) abnormality after chemotherapy containing etoposide for langerhans' cell histiocytosis Juan A. Lopez-Andreu M.D., Juan A. Lopez-Andreu M.D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorJosep Ferrís Ph. D., Josep Ferrís Ph. D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorAmparo Verdeguer M.D., Amparo Verdeguer M.D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorCarlos Esquembre M.D., Carlos Esquembre M.D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorVictoria Castel Ph. D., Victoria Castel Ph. D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorKeizo Horibe M.D., Keizo Horibe M.D. Department of Pediatrics, Nogoya University School of Medicine Nagoya, JapanSearch for more papers by this author Juan A. Lopez-Andreu M.D., Juan A. Lopez-Andreu M.D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorJosep Ferrís Ph. D., Josep Ferrís Ph. D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorAmparo Verdeguer M.D., Amparo Verdeguer M.D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorCarlos Esquembre M.D., Carlos Esquembre M.D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorVictoria Castel Ph. D., Victoria Castel Ph. D. Hospital Infantil La Fé Unidad de Oncología Pediátrica Valencia, SpainSearch for more papers by this authorKeizo Horibe M.D., Keizo Horibe M.D. Department of Pediatrics, Nogoya University School of Medicine Nagoya, JapanSearch for more papers by this author First published: 1 September 1994 https://doi.org/10.1002/1097-0142(19940901)74:5<1650::AID-CNCR2820740528>3.0.CO;2-UCitations: 2AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume74, Issue51 September 1994Pages 1650-1652 ReferencesRelatedInformation

Treatment strategy for disseminated langerhans cell histiocytosis

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.

Treatment of Langerhans cell histiocytosis in children with etoposide.

Ten children with Langerhans cell histiocytosis (LCH) were treated with etoposide. For five patients, this was the initial diagnosis. The other five had failed to respond to previous therapies. Etoposide (100 mg/m2) was given intravenously twice a week for 4 weeks, followed by maintenance therapy every 2 to 4 weeks for 2 years. All 10 patients responded to etoposide, and 6 of them (60%) have been in complete remission for 3 to 36 months without any side effects. One patient relapsed with diabetes insipidus, one with a soft tissue mass, and two others developed multiple bone lesions. Chemotherapy with etoposide appears to be effective and safe for the treatment of children with systemic LCH.

Etoposide in the treatment of six children with Langerhans cell histiocytosis (histiocytosis X)

Six children received etoposide as the single agent for treatment of Langerhans cell histiocytosis (LCH; histiocytosis X). Five were less than 2 years old at diagnosis. All had multiorgan involvement; one had liver and pulmonary dysfunction. Two infants also had clinical signs of immune deficiency. Complete response was observed in five. There was no major toxicity. Although three of four evaluable patients relapsed, the drug was considered useful in moving the children from a symptomatic to an asymptomatic clinical status. Etoposide may become a "first-line" drug in the treatment of systemic LCH, especially when the side effects of steroid therapy are considered unacceptable.