Clinical Characteristics and Outcome of Young Chronic Lymphocytic Leukemia Patients: A Single Institution Study of 204 Cases

Abstract

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.

Modern and conventional prognostic markers of chronic lymphocytic leukaemia in the everyday haematological practice

“…In patients with CLL, we found that a 1-yr increase in age led to approximately a 6% increase in the risk of death (when the patients who died from causes other than CLL were excluded from our analysis, a 1-yr increase in age led to a 5% increase in the risk of death). This finding implies that the prognosis of patients with CLL is not only affected by the disease itself but also by associated comorbidities (14). Our results show an evident difference in the OS of the entire group (148 months) from that of patients who died of causes unrelated to CLL (cardiovascular disease, other malignancy and accident; all infections were considered as CLL related in our analysis) (227 months).…”

Stem cell transplantation for chronic lymphocytic leukaemia

“…About a third of CLL patients are under the age of 60 years and 10-15% are younger than 50 years. In young adults, CLL has no major distinctive features, the prognostic factors are the same as those in older patients and median survival is less than 3 years for young patients with advanced CLL (Mauro et al, 1999). Young patients with poor risk CLL will need aggressive therapy aimed at achieving a cure, as standard treatment with alkylating agents or purine analogues offers a poor outlook.…”

Richter syndrome in B‐cell chronic lymphocytic leukemia

“…reviewed 1011 patients with B-CLL, in which 22 patients terminated to RS (2.2%). 12 These patients constituted 18 cases with DLBCL (1.8%) and four cases with HL (0.4%). A different frequency of RS in a younger group from that in an older group was found.…”

“…75 Mauro et al reported 22 patients with RS among 1011 B-CLL, which consisted of 18 cases with DLBCL and four cases with HL. 12 The rate of patients with B-CLL who developed in HL might account for approximately 0.5% of cases. The report of long-term follow-up of patients with B-CLL receiving Fludarabine regimens as initial therapy, which were produced from the same institution to Fayad et al, revealed four cases of RS found in 174 cases of B-CLL (2.3%).…”

“…4 There have been a lot of case studies and reviews about RS. [5][6][7][8][9][10][11][12][13] A concept of RS has been well built, but an effective therapy that can cure the patient with RS is not available. In addition, RS is known to evolve in the natural course of B-CLL without prior chemotherapy, and both RS related to clonal evolution 14,15 and RS unrelated to the original 15 clone have been reported.…”