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: 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 10/2008; 52(1):11-3. · 2.35 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 12/2009; 54(4):603-5. · 2.35 Impact Factor