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

Clin Lymphoma Myeloma Leuk. 2020 Jun;20(6):e270-e283. doi: 10.1016/j.clml.2019.11.002. Epub 2019 Nov 7.

Abstract

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.

Keywords: 17p Deletion; B-CLPDs; Differential diagnosis; Distribution; Universal prognostic factors.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • B-Lymphocytes*
  • China / epidemiology
  • Chromosome Deletion*
  • Chromosomes, Human / genetics*
  • Chronic Disease
  • Disease-Free Survival
  • Female
  • Humans
  • Lymphoproliferative Disorders* / genetics
  • Lymphoproliferative Disorders* / mortality
  • Male
  • Middle Aged
  • Risk Factors
  • Survival Rate