Isolated central nervous system relapse in childhood acute promyelocytic leukemia.
ABSTRACT Central nervous system (CNS) involvement is rare in acute promyelocytic leukemia (APL). The majority of CNS relapses occur in patients with hyperleukocytosis at presentation, and the optimal management of such patients is still controversial. We describe a 13-year-old boy with APL who developed an isolated CNS relapse after first-line treatment with all-trans retinoic acid and chemotherapy. A second remission was achieved with a regimen consisting of intrathecal chemotherapy, intravenous high-dose cytarabine, and oral 6-mercaptopurine. All-trans retinoic acid was avoided owing to severe complications during initial therapy. The patient remains in molecular remission at 9 months after autologous stem cell transplant. Prognostic factors of CNS relapse in children with APL are needed to define the indications for CNS prophylaxis in this group of patients.
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ABSTRACT: RESUMEN La leucemia aguda promielocítica se distingue del resto de las leucemias mieloblásticas en la biología, clínica, pronóstico y tratamiento. Se reconocen tres grupos de riesgo. La frecuencia de infiltración al sistema nervioso central es baja. Se reporta una serie de cuatro casos con leucemia aguda promielocítica y recaída al sistema nervioso central. Tres pacientes son adultos y uno pediátrico. Dos pacientes son de alto riesgo, uno con riesgo intermedio y el otro con riesgo bajo. En la inducción a la remisión todos los pacientes se trataron con ácido holo-transretinoico y antraciclinas y se continuó con consolidaciones dependiendo riesgo. En uno de los pacientes se utilizó trióxido de arsénico y trasplante autólogo posterior a la recaída. Se revisa la bibliografía y se concluye que en pacientes con riesgo alto debe consi-derarse la profilaxis para leucemia meníngea. Palabras clave: promielocítica, leucemia, infiltración meníngea , sistema nervioso central, aguda. ABSTRACT Acute promyelocytic leukemia (APL) is distinguished from other myeloblastic leukemias in biology, clinical behavior, prognosis and treatment. Three risk groups are recognized. The frequency of infiltration into central nervous system (CNS) is low. We present a series of 4 cases LAP with CNS relapse. Three adults and one child. Two patients were of high risk, one intermediate risk and other had a low risk. All were treated with all-trans retinoic acid (ATRA) and anthracyclines as induction therapy, and consolidation was continued according to risk. In one patient we used arsenic trioxide and autologous transplantation after relapse. A review of the literature is presented and we concluded that in patients at high risk , prophylaxis of meningeal leukemia should be administered.
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ABSTRACT: The incidence of isolated central nervous system (iCNS) relapse in pediatric acute promyelocytic leukemia (APL) is debated. We analyzed the literature, focusing on clinical trials reported since the advent of ATRA use. Only 2/218 (0.92%) good risk patients (diagnostic WBC <10,000/microl) had truly iCNS relapse. This incidence does not support the use of intrathecal CNS prophylaxis for all children with APL. We also identified multiple deficiencies in these reports. Additional reporting of these events could provide insight into the true incidence and pathogenesis of iCNS relapse and might allow for identification of risk factors associated with such extramedullary relapse.Pediatric Blood & Cancer 01/2009; 52(1):11-3. DOI:10.1002/pbc.21608 · 2.56 Impact Factor
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ABSTRACT: Central nervous system (CNS) involvement is rarely observed in acute promyelocytic leukemia (APML). Most cases of CNS involvement occur at relapse rather than at presentation. Because of the extremely low incidence of CNS disease, diagnostic lumbar puncture is not routinely required and prophylactic intrathecal chemotherapy is not routinely administered. Here, we describe a teenage patient with newly diagnosed APML, chloromas, and symptomatic CNS involvement confirmed by MRI and cerebrospinal fluid (CSF) findings.Pediatric Blood & Cancer 01/2009; 54(4):603-5. DOI:10.1002/pbc.22348 · 2.56 Impact Factor