Staging and treatment
Two staging systems are used to determine disease stage in patients with CLL, both of which include factors such as hemoglobin level, platelet count, and lymphocytosis/lymphadenopathy.3 The Binet system categorizes patients as clinical stage A, B, and C, whereas the Rai system categorizes patients into stage 0 to IV.3 With both systems, higher stage indicates worse disease.3 Because most cases of CLL are “incurable,” treatment goals are to prolong survival and maintain or improve quality of life.3
Patients with early disease
Persons who have early-stage CLL and are asymptomatic (Binet stage A and B without active disease; Rai 0, I, and II without active disease) are typically followed with regular monitoring of symptoms and laboratory testing.3,5 Treatment begins if there are signs of disease progression, such as development of chromosomal abnormalities or changes in blood cell counts (eg, hemoglobin, white blood cells, platelets), bone marrow or lymph node morphology, or serum markers (eg, high serum beta-2 microglobulin).3,5
Patients with advanced disease
Treatment is recommended for persons with advanced disease (Binet stage A or B with active disease or stage C; Rai 0-II with active disease or Rai III-IV).3,5 Active disease is defined by the presence of at least 1 of the following3:
- Progressive bone marrow failure (development or worsening of anemia/thrombocytopenia)
- Massive, progressive, or symptomatic splenomegaly
- Massive, progressive, or symptomatic lymphadenopathy
- Progressive lymphocytosis
- Autoimmune complications (eg, thrombocytopenia not responsive to corticosteroids)
- Extranodal involvement (eg, kidney, lung)
- Disease-related symptoms, such as ≥10% weight loss in the past 6 months
Patients with CCL may be initially tested for IGHV and TP53 gene mutation status, del(17p), del(11q), serum beta-2 microglobulin levels, and cell surface markers CD38, CD49d, and ZAP-70. All can influence treatment choices, which include tyrosine kinase inhibitors, B-cell receptor inhibitors, and immune checkpoint inhibitors.3,5,8,9 Treatment selection also takes into account factors such as other cytogenetic abnormalities, the patient’s physical status, medications, comorbidities, and treatment adherence.3,5