title: Central nervous system involvement in adult acute lymphoblastic leukemia: diagnostic tools, prophylaxis, and therapy.
abstract: In adult patients with acute lymphoblastic leukemia (ALL), Central Nervous System (CNS) involvement is associated with a very poor prognosis. The diagnostic assessment of this condition relies on the use of neuroradiology, conventional cytology (CC) and flow cytometry (FCM). Among these approaches, which is the gold standard it is still a matter of debate. Neuroradiology and CC have a limited sensitivity with a higher rate of false negative results. FCM demonstrated a superior sensitivity over CC, particularly when low levels of CNS infiltrating cells are present. Although prospective studies of a large series of patients are still awaited, a positive finding by FCM appears to anticipate an adverse outcome even if CC shows no infiltration. Current strategies for adult ALL CNS-directed prophylaxis or therapy involve systemic and intrathecal chemotherapy and radiation therapy. An early and frequent intrathecal injection of cytostatic combined with systemic chemotherapy is the most effective strategy to reduce the frequency of CNS involvement. In patients with CNS overt ALL, at diagnosis or upon relapse, allogeneic hematopoietic stem cell transplantation might be considered. This review discusses risk factors, diagnostic techniques for identification of CNS infiltration and modalities of prophylaxis and therapy to manage it.
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Maria Ilaria
authors.initials: MI
authors.lastname: Del Principe
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Luca
authors.initials: L
authors.lastname: Maurillo
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Francesco
authors.initials: F
authors.lastname: Buccisano
authors.affiliation: Istituto di Farmacologia Translazionale, Dipartimento di Medicina, CNR, Roma, Italia.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Sconocchia
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Mariagiovanna
authors.initials: M
authors.lastname: Cefalo
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Giovanna
authors.initials: G
authors.lastname: De Santis
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Ambra
authors.initials: A
authors.lastname: Di Veroli
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Concetta
authors.initials: C
authors.lastname: Ditto
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Daniela
authors.initials: D
authors.lastname: Nasso
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Postorino
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Marco
authors.initials: M
authors.lastname: Refrigeri
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Cristina
authors.initials: C
authors.lastname: Attrotto
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Del Poeta
authors.affiliation: Fondazione S. Lucia.
authors.forename: Francesco
authors.initials: F
authors.lastname: Lo-Coco
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Sergio
authors.initials: S
authors.lastname: Amadori
authors.affiliation: Ematologia, Dipartimento di Biomedicina e Prevenzione, Università Tor Vergata, Roma, Italia.
authors.forename: Adriano
authors.initials: A
authors.lastname: Venditti