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Blood, Vol. 94 No. 2 (July 15), 1999:
pp. 448-454
Clinical Characteristics and Outcome of Young Chronic Lymphocytic
Leukemia Patients: A Single Institution Study of 204 Cases
Francesca R. Mauro,
Robert Foa,
Diana Giannarelli,
Iole Cordone,
Sabrina Crescenzi,
Edoardo Pescarmona,
Roberta Sala,
Raffaella Cerretti, and
Franco Mandelli
From the Dipartimento di Biotecnologie Cellulari ed Ematologia and
Dipartimento di Medicina Sperimentale e Patologia, University "La
Sapienza" of Rome, Rome, Italy; and the Dipartimento di Scienze
Biomediche ed Oncologia Umana, University of Turin, Turin, Italy.
A retrospective analysis on chronic lymphocytic leukemia (CLL)
patients  55 years observed at a single institution was performed with the purpose of characterizing the clinical features and outcome of
young CLL and of identifying patients with different prognostic features. Over the period from 1984 to 1994, 1,011 CLL patients (204 [20%] 55 years of age and 807 [80%] >55 years of age) were observed. At diagnosis, younger and older patients displayed a similar
distribution of clinical features, except for a significantly higher
male/female ratio in younger patients (2.85 v 1.29;
P < .0001). Both groups showed an elevated rate of
second primary cancers (8.3% v 10.7%), whereas the occurrence
of Richter's syndrome was significantly higher in younger patients
(5.9% v 1.2%; P < .00001). Younger and older
patients showed a similar overall median survival probability (10 years) but were characterized by a different distribution of causes of
deaths: CLL unrelated deaths and second primary malignancies
predominated in the older age group, whereas the direct effects of
leukemia were prevalent in the younger age group. Although younger and
older patients displayed a similar survival, the evaluation of the
relative survival rates showed that the disease had a greater adverse
effect on the expected survival probability of the younger population.
Multivariate analysis showed that for young CLL patients only dynamic
parameters, such as lymphocyte doubling time and other signs of active
disease, were the independent factors that significantly influenced
survival probability (P = .00001). A prolonged
clinico-hematologic follow-up allowed us to identify two subsets of
young CLL patients with a different prognostic outcome: a group of
patients (40%) with long-lasting stable disease without treatment and
an actuarial survival probability of 94% at 12 years from diagnosis
and another group (60%) with progressive disease and a median survival
probability of 5 years after therapy. For the latter patients, the
therapeutic effect of innovative therapies with curative intents needs
to be investigated in prospective, comparative clinical trials.
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