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1.
Br J Haematol ; 169(5): 711-8, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25817915

RESUMEN

Patients with Langerhans cell histiocytosis (LCH) refractory to conventional chemotherapy have a poor outcome. There are currently two promising treatment strategies for high-risk patients: the first involves the combination of 2-chlorodeoxyadenosine and cytarabine; the other approach is allogeneic haematopoietic stem cell transplantation (HSCT). Here we evaluated 87 patients with high-risk LCH who were transplanted between 1990 and 2013. Prior to the year 2000, most patients underwent HSCT following myeloablative conditioning (MAC): only 5 of 20 patients (25%) survived with a high rate (55%) of transplant-related mortality (TRM). After the year 2000 an increasing number of patients underwent HSCT with reduced intensity conditioning (RIC): 49/67 (73%) patients survived, however, the improved survival was not overtly achieved by the introduction of RIC regimens with similar 3-year probability of survival after MAC (77%) and RIC transplantation (71%). There was no significant difference in TRM by conditioning regimen intensity but relapse rates were higher after RIC compared to MAC regimens (28% vs. 8%, P = 0·02), although most patients relapsing after RIC transplantation could be salvaged with further chemotherapy. HSCT may be a curative approach in 3 out of 4 patients with high risk LCH refractory to chemotherapy: the optimal choice of HSCT conditioning remains uncertain.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Histiocitosis de Células de Langerhans/terapia , Acondicionamiento Pretrasplante , Adolescente , Adulto , Niño , Preescolar , Femenino , Enfermedad Injerto contra Huésped/etiología , Enfermedad Injerto contra Huésped/prevención & control , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Histiocitosis de Células de Langerhans/mortalidad , Histiocitosis de Células de Langerhans/patología , Humanos , Lactante , Masculino , Estudios Retrospectivos , Trasplante Homólogo , Resultado del Tratamiento , Adulto Joven
2.
Biol Blood Marrow Transplant ; 20(11): 1847-51, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25016194

RESUMEN

Mucolipidosis type II (MLII), or I-cell disease, is a rare but severe disorder affecting localization of enzymes to the lysosome, generally resulting in death before the 10th birthday. Although hematopoietic stem cell transplantation (HSCT) has been used to successfully treat some lysosomal storage diseases, only 2 cases have been reported on the use of HSCT to treat MLII. For the first time, we describe the combined international experience in the use of HSCT for MLII in 22 patients. Although 95% of the patients engrafted, overall survival was low, with only 6 patients (27%) alive at last follow-up. The most common cause of death post-transplant was cardiovascular complications, most likely due to disease progression. Survivors were globally delayed in development and often required complex medical support, such as gastrostomy tubes for nutrition and tracheostomy with mechanical ventilation. Although HSCT has demonstrated efficacy in treating some lysosomal storage disorders, the neurologic outcome and survival for patents with MLII were poor. Therefore, new medical and cellular therapies should be sought for these patients.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas/métodos , Mucolipidosis/terapia , Acondicionamiento Pretrasplante/métodos , Preescolar , Recolección de Datos , Humanos , Lactante , Encuestas y Cuestionarios , Resultado del Tratamiento
3.
Transplantation ; 78(5): 755-7, 2004 Sep 15.
Artículo en Inglés | MEDLINE | ID: mdl-15371682

RESUMEN

Epstein-Barr virus (EBV) lymphoproliferative disease (LPD) is a potentially fatal complication that may follow allogeneic hematopoietic stem-cell transplantation (HSCT). In this article, the authors report a 2-year-old girl with Hurler's syndrome who developed multiple central nervous system (CNS) EBV LPD lesions 1 year after unrelated donor HSCT. Before this CNS occurrence, the patient had a complete response to rituximab treatment for EBV LPD of the spleen and lymph nodes; however, treatment of the CNS disease with rituximab proved ineffective. Because of reported favorable response of primary CNS EBV LPD in two human immunodeficiency virus-positive patients, the authors treated this patient with low-dose oral hydroxyurea. The patient improved clinically, with a decrease in size of multiple EBV LPD brain lesions. Subsequently, the patient received EBV-specific cytotoxic T-cell lymphocytes and remains well. The benefit and limited toxicity of hydroxyurea therapy merit its further consideration as treatment for EBV LPD.


Asunto(s)
Infecciones por Virus de Epstein-Barr/etiología , Transfusión de Linfocitos/efectos adversos , Trastornos Linfoproliferativos/etiología , Trasplante de Células Madre/efectos adversos , Adulto , Encéfalo/diagnóstico por imagen , Preescolar , Humanos , Hidroxiurea , Mucopolisacaridosis I/terapia , Linfocitos T/trasplante , Linfocitos T Citotóxicos/inmunología , Tomografía Computarizada por Rayos X
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