Chronic lymphocytic leukemia (CLL)
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Clinically heterogeneous chronic lymphocytic leukemia is the most common leukemic disease in Germany. Based on the current guidelines and the current state of research, there are different diagnostic recommendations for patients with CLL. We have summarized the most important information on classification and present the diagnostic methods at MLL. In addition, we provide further links on prognosis and therapy in CLL, so that you can inform yourself in more detail.
Chronic lymphocytic leukemia: Classification
The WHO classification 2022 assigns chronic lymphocytic leukemia (CLL) to mature B-cell neoplasms. Together with monoclonal B-cell lymphocytosis (MBL), CLL makes up the group of 'pre-neoplastic and neoplastic small cell lymphoid proliferative diseases' (WHO 2022). CLL is an indolent lymphocytic lymphoma characterized by a leukemic course. Differentiation from other lymphoma entities must be done by immunophenotyping, cytomorphology, FISH, and histology.
Chronic lymphocytic leukemia: diagnostic methods and their significance
Chronic lymphocytic leukemia: Prognosis
Genetic characteristics are important prognostic factors and are considered in the context of various scores
A complex karyotype, IGHV status, and TP53 aberrations represent important prognostic markers in CLL.
IGHV and TP53 status are included in the CLL-IPI (International Prognostic Index). For this, the parameters age, Binet stage and serum ß2-microglobulin are also taken into account. For asymptomatic early-stage CLL, the international prognostic score (IPS-E) allows estimation of time to first treatment. This score includes only three risk factors (unmutated IGHV status, lymphocytes ≥15 x109 /L, lymph node involvement) (Condoluci et al. 2020). The Rossi et al. 2013 prognostic panel considers both molecular genetic (TP53 and BIRC3 aberrations, NOTCH1 and SF3B1 mutations) and cytogenetic markers (del(11q), +12, normal karyotype, isolated del(13q)) and can be applied both at diagnosis and during progression.
Chronic lymphocytic leukemia: Prognosis calculation
Chronic lymphocytic leukemia: Therapy
Predictive markers that determine first-line therapy are IGHV mutation status, TP53 aberrations, and complex karyotype. Details on therapy according to the German guideline, which provides an up-to-date overview of current treatment options with BTK inhibitors, anti-CD20 monoclonal antibodies, and the BCL-2 inhibitor venetoclax, can be found on the Onkopedia pages.
Chronic lymphocytic leukemia: Recommendations
If CLL is suspected in a patient, differential blood counts and immunophenotyping are always indicated for guideline-compliant diagnosis. The recommendations for genetic testing are summarized in Table 2.
Table 2: Diagnostic recommendations in various guidelines
S3 guideline |
Shall: FISH for del(17p); TP53 analysis Should: IGHV status; FISH for del(11q22) Can: del(6q21~q23), del(13q14), +12, karyotyping |
iwCLL guideline |
Always: FISH on del(13q), del (11q), del(17p) and +12; TP53 and IGHV status Desirable: Karyotyping |
Onkopedia guideline |
FISH for del(17p); TP53 and IGHV status; testing for a complex karyotype; further genetic analysis for atypical CLL phenotype |
MRD diagnosis is currently listed as a "can" recommendation (S3 guideline 2018). In the case of time-limited therapy, MRD determination is recommended at the earliest 2 months after the end of therapy; in the case of continuous therapy, the time of best response should be selected, regardless of whether complete or partial remission has been achieved (Wierda et al. 2021).
Status: September 2023