Incidence and risk factors for central nervous system relapse in children and adolescents with acute lymphoblastic leukemia

Abstract

BackgroundDespite all the advances in the treatment of childhood acute lymphoblastic leukemia, central nervous system relapse remains an important obstacle to curing these patients. This study analyzed the incidence of central nervous system relapse and the risk factors for its occurrence in children and adolescents with acute lymphoblastic leukemia. Methods This study has a retrospective cohort design. The studied population comprised 199 children and adolescents with a diagnosis of acute lymphoblastic leukemia followed up at Hospital das Clinicas, Universidade Federal de Minas Gerais (HC-UFMG) between March 2001 and August 2009 and submitted to the Grupo Brasileiro de Tratamento de Leucemia da Infância - acute lymphoblastic leukemia (GBTLI-LLA-99) treatment protocol. Results The estimated probabilities of overall survival and event free survival at 5 years were 69.5% (± 3.6%) and 58.8% (± 4.0%), respectively. The cumulative incidence of central nervous system (isolated or combined) relapse was 11.0% at 8 years. The estimated rate of isolated central nervous system relapse at 8 years was 6.8%. In patients with a blood leukocyte count at diagnosis ≥ 50 x 109/L, the estimated rate of isolated or combined central nervous system relapse was higher than in the group with a count < 50 x 109/L (p-value = 0.0008). There was no difference in cumulative central nervous system relapse (isolated or combined) for the other analyzed variables: immunophenotype, traumatic lumbar puncture, interval between diagnosis and first lumbar puncture and place where the procedure was performed. Conclusions These results suggest that a leukocyte count > 50 x 109/L at diagnosis seems to be a significant prognostic factor for a higher incidence of central nervous system relapse in childhood acute lymphoblastic leukemia.

Clinical characteristics of patients with central nervous system relapse in BCR-ABL1-positive acute lymphoblastic leukemia: the importance of characterizing ABL1 mutations in cerebrospinal fluid

“…Notably, it is somewhat age-dependent, and its frequency is higher than in other B cell precursor ALL diseases. In pediatric and adolescent populations, CNS recurrence occurs in 3–11% of cases [15–18]. These two series of middle-aged (<55 years) and older (>55 years) adults showed CNS relapse frequencies of 7 and 14%, respectively, which is in agreement with the findings of other groups [19–21] .…”

A Case of Acute Lymphoblastic Leukemia with Isolated Orbital Relapse

“…In ALL patients, like our case, hyperleukocytosis at administration, Philadelphia chromosome positivity show poor prognosis. The most frequent extramedullary involvement is seen in central nervous system (2). Orbital involvement is rarely seen in leukemia patients.…”

“…Acute lymphoblastic leukemia (ALL) is the most common malignancy of childhood, which could be curable in 80% with intensive chemotherapy protocols (1). Central nervous system is the most frequent place of both at the onset and relapse of the disease (2). Orbital involvement is rarely seen.…”

Steroid‐induced ocular hypertensive response in children and adolescents with acute lymphoblastic leukemia and non‐hodgkin lymphoma

“…The treatment protocols included high doses of GC (prednisone 40 mg/m 2 /day or Dexamethasone 6 mg/m 2 /day) for 28 days, with another 5 days of decreasing doses until complete withdrawal, during the remission induction phase, in three 7‐day cycles during the consolidation phase and in 7‐day cycles in the maintenance phase, resulting in at least 98 days of GC use during the entire treatment (prednisone 4 g/m 2 or Dexamethasone 600 mg/m 2 ) .…”

“…Acute lymphoblastic leukemia (ALL) is the most common cancer found among young people and, if analyzed together with non‐Hodgkin leukemia (NHL), one finds that they account for at least one third of the cases of diagnosed childhood cancer . Present‐day therapeutic protocols include high dosages of glucocorticoids (GC) , drug associated with high potential for elevating intraocular pressure (IOP) and, consequently, damaging the fibers of the optic nerve (cortisone glaucoma) .Cortisone glaucoma occurs in genetically susceptible patients during the period of administration of GC and is generally reversible with the suspension of its use. However, depending on the levels of IOP reached and the duration of ocular hypertension, damage can result in definitive optic neuropathy and even blindness .…”