rome and Precursor-Cell-Lymphoblastic-Leukemia-Lymphoma

Topics: Allopurinol; Child; Humans; Maintenance; Mercaptopurine; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Rome

We analyzed 12 MLL/ENL positive ALL patients consecutively diagnosed between 1999 and 2009. The MLL/ENL fusion was identified in 4/150 (2.6%), 8/993 (0.8%), and 0/70 of pediatric, adult, and elderly patients, respectively. Eight patients had a WBC count >50 × 10(9) /L. Ten cases had an evaluable immunophenotyping. A B or T precursor ALL occurred in 7 and 3 patients, respectively. Eleven/12 patients (92%) achieved CR. At 48 months, overall survival and event-free survival rates were 73.3% and 67%, respectively. At CR, a parallel RT-PCR evaluation of the MLL/ENL expression was available in 5 cases. Of these latter, 2 tested MLL/ENL-negative and 3 positive. The minimal residual disease molecular monitoring showed that MLL/ENL status did not correlate with outcome. In fact, all the 2 PCR-negative and 1 of the 3 PCR-positive cases relapsed. Further, a MLL/ENL expression, not preceding a relapse, was detected several times during the follow-up of five long-survivors. In conclusion, also in adults, the MLL/ENL fusion identifies a rare leukemic entity with a favorable prognosis. The observed inconsistency between the clinical cure and the presence of detectable MLL/ENL transcript suggests the existence of a MLL/ENL-expressing "preleukemia" stem cells, similar to what demonstrated for the AML1/ETO-positive leukemia setting.

Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Cohort Studies; Female; Follow-Up Studies; Gene Expression Regulation, Leukemic; Humans; Infant; Male; Middle Aged; Myeloid-Lymphoid Leukemia Protein; Neoplasm, Residual; Oncogene Proteins, Fusion; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Prognosis; Remission Induction; Rome; Survival Analysis; Transcriptional Elongation Factors; Young Adult

The history of the treatment of childhood acute leukemia is a meaningful model of the ethical, bioethical and organizational implications of the technical progress in medicine. This experience provides indications and very useful tools to face the main topics of modern medicine: the risk of intense medicalization to the detriment of the quality of the care, the importance of psycho-emotive, ethical and spiritual aspects in the doctor-patient relationship, etc. The Authors report the difficulties and progresses until and after the first cures of children with leukemia in the Pediatric Clinic of the University "La Sapienza" of Rome (1964), the new features of treated leukemia (the real disease of cured child") and the psycho-emotive involvement of the pediatric staff a "parallel disease"). The necessity of a continuous activity aimed at the adaptation of services to scientific and medical progress and to the necessity of humanization of procedures and environment is stressed.

Topics: Child; Clinical Trials as Topic; Disease Management; Female; Hematology; History, 20th Century; History, 21st Century; Hospitals, University; Humans; Male; Outpatient Clinics, Hospital; Pain; Parents; Pediatrics; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Rome; Societies, Medical; Stress, Psychological