Subtype Distribution, Clinical Features, and Survival in B-cell Chronic Lymphoproliferative Disorders in China: A Review of 1592 Cases.

BACKGROUND

B-cell chronic lymphoproliferative disorders (B-CLPDs) are characterized by the sustained accumulation of monoclonal B cells. Limited studies have systematically described the clinical features and outcomes of the whole patient group, especially in Eastern populations.

PATIENTS AND

METHODS

A total of 1592 patients with newly diagnosed B-CLPD were enrolled. Chronic lymphocytic leukemia (CLL) accounted for 39%, and Waldenström macroglobulinemia (WM), leukemic marginal zone lymphoma, follicular lymphoma (FL), and mantle cell lymphoma (MCL) constituted 13%, 13%, 9%, and 8% of cases, respectively.

RESULTS

The median age at diagnosis was 58 years, and the male/female ratio was 1.8:1. The 17p and 11q deletions were most common in MCL (36% and 17%, respectively), and 13q deletion and trisomy 12 were most frequent in CLL (35% and 21%, respectively). Patients with leukemic MCL had significantly worse survival than that of patients with other disease entities, with a 3-year overall survival (OS) of 58%, followed by 68.2% for WM/lymphoplasmacytic lymphoma. Those with CLL, leukemic marginal zone lymphoma, and FL had relatively favorable outcomes, with a 5-year OS > 80%. The survival of patients with B-CLPDs has improved over time with the emergence of novel drugs (3-year OS improvement from 82.1% to 92.2%). The improvement in survival mainly resulted from improvement among patients with MCL, WM/lymphoplasmacytic lymphoma, and FL. On multivariate analysis, only hemoglobin, lactate dehydrogenase, and 17p deletion were independently associated with survival (hazard ratio, 1.6, 2.0, and 3.1, respectively).

CONCLUSIONS

Comprehensive analysis of the clinical characteristics, immunophenotypic profiles, and cytogenetic features can be helpful in the differential diagnosis, especially for patients without a non-bone marrow biopsy specimen available. Universal prognostic factors could help with the early detection of high-risk patients and stratification for risk-adapted therapy.