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CENTRAL NERVOUS SYSTEM INVOLVEMENT IN ADULT ACUTE LYMPHOBLASTIC LEUKEMIA: DIAGNOSTIC TOOLS, PROPHYLAXIS AND THERAPY
Maria Ilaria Del Principe, Luca Maurillo, Francesco Buccisano, Giuseppe Sconocchia, Mariagiovanna Cefalo, Giovanna De Santis, Ambra Di Veroli, Concetta Ditto, Daniela Nasso, Massimiliano Postorino, Marco Refrigeri, Cristina Attrotto, Giovanni Del Poeta, Sergio Amadori, Adriano Venditti
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- Maria Ilaria Del Principe
Ematologia, Dipartimento di Biomedicina e Prevenzione Università Tor Vergata, Roma, Italia., Italy
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- Luca Maurillo
Affiliation not present
- Francesco Buccisano
Affiliation not present
- Giuseppe Sconocchia
Affiliation not present
- Mariagiovanna Cefalo
Affiliation not present
- Giovanna De Santis
Affiliation not present
- Ambra Di Veroli
Affiliation not present
- Concetta Ditto
Affiliation not present
- Daniela Nasso
Affiliation not present
- Massimiliano Postorino
Affiliation not present
- Marco Refrigeri
Affiliation not present
- Cristina Attrotto
Affiliation not present
- Giovanni Del Poeta
Affiliation not present
- Sergio Amadori
Affiliation not present
- Adriano Venditti
Affiliation not present
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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 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. Actually, 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, allogenic hematopoietic stem cell transplantation might be considered. This review will discuss risk factors, diagnostic techniques for identification of CNS infiltration and modalities of prophylaxis and therapy to manage it.
Keywords
acute lymphoblastic leukemia, CNS involvement, flow cytometry, intrathecal therapy.
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