title: Chronic Lymphocytic Leukemia in Younger Adults: Preliminary Results of a Study Based on 454 Patients-IWCLL/Working Group.
abstract: Whether all younger adult patients with chronic lymphocytic leukemia (CLL) should be offered intensive and/or experimental treatments is currently undecided. In order to investigate the natural history and prognosis of CLL in younger adults, information on 454 B-CLL patients less than 50 yrs old, from 13 different International groups was collected and analyzed. The male/female proportion is 1.46 (270/184) and the median age is 46 yrs (range, 20-49). Number of cases increases with age: nine patients (2%) are <30 yrs; 70 (14%) are 30 to 40 yrs; and 375 (84%) are 41 to 49 yrs. Clinical stages distribution is as follows: Rai O, 113 (25%), Rai I, 98 (21.5%), Rai II, 182 (40%), Rai III, 25 (5.5%), and Rai IV, 36 (8%); Binet's A, 235 (52%), Binet's B, 160 (35%), Binet's C 59 (13%). Bone marrow biopsy was available in 251 cases, being nodular in 46 (18.5%), interstitial in 76 (30%), mixed in 60 (24%), and diffuse in 69 (27.5%). Clinical characteristics of these patients do not differ from those described for entire series of CLL patients including all ages. The majority of patients (93%) were diagnosed between 1975 and 1989 and at the time of this analysis 138 (30.3%) have died. Infection accounts for the large majority of deaths (48%). Overall median survival is 12 yrs. Survival probability (±SD) is 73.55 ± 2.47% at 5 yrs, 51.04 ± 3.46% at 10 yrs, and 45.53 ± 4.08% at 15 yrs. Prognostic parameter identified in whole series of patients with CLL are also useful when applied to this special subset. Thus, patients in stage A, B, and C have, at 10 yrs after diagnosis, survival probabilities of 72.4%, 33.7%, and 21.2%, respectively. Rai stages also discriminate different risk-groups with a survival probability at 10 yrs of 76% for patients in stage 0, 45% for those in stage I + II, and 24% for patient in Stage III + IV. Patients with non-diffuse bone marrow pattern have a survival probability of 76% at 10 yrs compared to 28% for those with a diffuse pattern. Further analysis will refine both the prognostic assessment and the optimal treatment approach for these patients.
authors.affiliation: a Hellenic CLL Study Group, Italian Cooperative Group for CLL, Karolinska Institute Sweden, University of Rochester USA, Radcliffe Infirmary UK, Spanish Cooperative Group for CLL Study, Centre Henri Becquerel Rouen France, Centre Hospitalier et Universitaire de Rennes France, Centre Hospitalier Regional et Universitaire de Caen France, Centre Hospitalier Regional et Universitaire de Poitiers France, Hadassah University Hospital Jerusalem Israel, Rigshospitalet University Hospital Denmark.
authors.forename: G A
authors.initials: GA
authors.lastname: Pangalis
authors.affiliation: a Hellenic CLL Study Group, Italian Cooperative Group for CLL, Karolinska Institute Sweden, University of Rochester USA, Radcliffe Infirmary UK, Spanish Cooperative Group for CLL Study, Centre Henri Becquerel Rouen France, Centre Hospitalier et Universitaire de Rennes France, Centre Hospitalier Regional et Universitaire de Caen France, Centre Hospitalier Regional et Universitaire de Poitiers France, Hadassah University Hospital Jerusalem Israel, Rigshospitalet University Hospital Denmark.
authors.forename: J C
authors.initials: JC
authors.lastname: Reverter
authors.affiliation: a Hellenic CLL Study Group, Italian Cooperative Group for CLL, Karolinska Institute Sweden, University of Rochester USA, Radcliffe Infirmary UK, Spanish Cooperative Group for CLL Study, Centre Henri Becquerel Rouen France, Centre Hospitalier et Universitaire de Rennes France, Centre Hospitalier Regional et Universitaire de Caen France, Centre Hospitalier Regional et Universitaire de Poitiers France, Hadassah University Hospital Jerusalem Israel, Rigshospitalet University Hospital Denmark.
authors.forename: V A
authors.initials: VA
authors.lastname: Boussiotis
authors.affiliation: a Hellenic CLL Study Group, Italian Cooperative Group for CLL, Karolinska Institute Sweden, University of Rochester USA, Radcliffe Infirmary UK, Spanish Cooperative Group for CLL Study, Centre Henri Becquerel Rouen France, Centre Hospitalier et Universitaire de Rennes France, Centre Hospitalier Regional et Universitaire de Caen France, Centre Hospitalier Regional et Universitaire de Poitiers France, Hadassah University Hospital Jerusalem Israel, Rigshospitalet University Hospital Denmark.
authors.forename: E
authors.initials: E
authors.lastname: Montserrat
keywords: CLL; lymphocytic leukemia; young adults