Chronic lymphocytic leukemia

Background

Epidemiology

• Most common leukemia in adults
• Older adults, median age 70, but can present in anyone over 30
• More often men than women (2:1)

Staging (RAI)

• Low risk (Stage 0): lymphocytosis alone
• Intermediate risk (Stage I-II): lymphocytosis with lymphadenopathy, with or without hepatosplenomegaly
• High risk (Stage III-IV): lymphocytosis with anemia or thrombocytopenia

Clinical Manifestations

• Usually feel well until incidentally discovered on CBC with lymphocytosis
• Can present with painless lymphadenopathy, especially cervical, that waxes and wanes
• Less than 10% present with consitutional symptoms
  • Weight loss >10% within the last 6 months
  • Fevers for 2 weeks without infection
  • Drenching night sweats
  • Fatigue
• On exam, often have lymphadenopathy (50-90%), splenomegaly, hepatomegaly, and leukemia cutis (skin involvement)

Complications

• Infection is the major complication and is a common cause of death
• Also at risk for other malignancies: skin cancer, prostate cancer, and breast cancer
• Can develop autoimmune cytopenias, most commonly an autoimmune hemolytic anemia
• Can transform to acute lymphoma, most commonly DLBCL (Richter's transformation) at a rate of 0.5% per year

Management

• Active surveillance is appropriat for most patients
• Primary indication for treatment is the presence of symptoms
  • Progressive bone marrow failure not due to autoimmune destruction
  • Massive or symptomatic splenomegaly (≥6 cm below the costal margin)
  • Massive or symptomatic lymphadenopathy (≥10 cm)
  • Autoimmune cytopenias not responsive to other treatment
  • Constitutional symptoms
• First-line: fludarabine, cyclophosphamide, and rituximab (FCR)
• If older with comorbidities: chlorambucil with obinutuzumab, or bendamustine with rituximab (BR)