Chronic lymphocytic leukemia
From IDWiki
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)
