Factors of an unfavourable prognosis in patients withB-cell chronic lymphoid leukemia: a retrospective analysisof 206 cases

Mm. To assess factors of an unfavourable prognosis in a group of intermediate risk of B-cell chronic
lymphoid leukemia (BCCLL).
Material and methods. 206 BCCLL patients (mean age 55.5 years, male/female = 1.66) entered the
study conducted by Hematological Research Center in 1992-2000.
Results. Nine patients under 35 years of age did not survive 5 years except one female who achieved a
complete remission on fludarabin. The type of bone marrow infiltration (diffuse vs interstitial and nodular),
the time of lymphocyte count doubling (under or over 12 months) discriminate the patients by
prognosis in the group of intermediate risk: medians of overall survival 65 months vs 148 months and
72 vs 133 months, respectively (p < 0.005 for both curves, log-rank criterion). Survival medians in
groups with low (< 50% cells) and high (> 50% cells) expression of CD38+ cells in the group of intermediate
BCCLL risk comprise 55 and 106 months (p = 0.005). The type of bone marrow infiltration
and time of doubling of lymphocyte count overlap: > 70% patients with a diffuse type of bone marrow
infiltration have the time of doubling under 12 months and vice versa while expression of CD38 do not
overlap with these values. Combination of two signs (type of bone marrow infiltration and CD38 expression
or time og lymphocyte count doubling and CD38 expression) allows more precise identification
of prognostically unfavourable groups. Medians of survival for combination of the first two signs
(two positive against two negative) comprise 51 months vs 169 months (p < 0.0001), for combination
of the latter two signs 55 months vs 106 months was not reached (p < 0.001). Although most patients
with a tumor form of BCCLL are referred to stage II, the prognosis in this form is much worse than in
stage II, survival medians are 44 and 69 months, respectively (p < 0.05). A mutation status of the
genes of a variable region of immunoglobulins enable identification of the group of patients with a relatively
benign course of BCCLL (survival medians 61 and 289 months, p < 0.0001).
Conclusion. In patients under 35 years of age BCCLL runs unfavourably and seems to require intensive
poly chemotherapy. Usage of a combination of the signs (CD38, time of doubling of lymphocyte
count and type of bone marrow infiltration) is a simple and reliable method of identification of prognostically
different categories of patients in the group of an intermediate BCCLL risk. Prognosis in patients
with a tumor form of BCCLL is unfavourable.medians of survival in patients with a tumor form
and stage III-IV are comparable. Mutational status of the genes of immunoglobulin variable region
may serve a marker of a long-term prognosis.

B-cell chronic lymphocytic leukemia, prognostic factors, CD38, IgV mutational status, CD38