Can Chronic Lymphocytic Leukemia (CLL) cause an enlarged spleen, also known as splenomegaly?

Splenomegaly in Chronic Lymphocytic Leukemia (CLL)

Yes, splenomegaly (enlarged spleen) is a common clinical manifestation of Chronic Lymphocytic Leukemia (CLL) and occurs in a significant percentage of patients with this disease.

Prevalence and Pathophysiology

• Children and adolescents with chronic leukemias present with a higher frequency of splenomegaly and larger spleen size compared to adult patients 1
• In CLL, splenomegaly is one of the key physical findings used in clinical staging systems and disease assessment 1
• Splenomegaly in CLL results from infiltration of leukemic cells into the spleen, causing enlargement and potential hypersplenism

Clinical Significance and Staging

• Splenomegaly is included as a key parameter in both major CLL staging systems:

  • Rai staging system: Palpable splenomegaly is a defining feature in certain stages
  • Binet staging system: Palpable spleen is counted as one of the five possible areas of involvement for staging purposes 1

• According to the Binet staging system:

  • Stage A: Up to 2 involved areas (may include spleen)
  • Stage B: 3 or more involved areas (often includes splenomegaly)
  • Stage C: Presence of anemia or thrombocytopenia regardless of spleen size 1

Clinical Presentation and Symptoms

• Splenomegaly in CLL can range from mild to massive enlargement

• Symptomatic splenomegaly may present with:

  • Left upper quadrant pain or discomfort
  • Early satiety due to compression of the stomach
  • Abdominal distension
  • In severe cases, splenic infarction or rupture (rare)

• When evaluating CLL patients, careful physical examination must rule out complications such as symptomatic splenomegaly 1

Treatment Considerations

• Splenomegaly that persists or increases despite tyrosine kinase inhibitor (TKI) therapy may indicate disease progression or transformation to accelerated phase 1

• Treatment options for symptomatic splenomegaly in CLL include:

  1. Systemic CLL therapy (first-line approach)
  2. Splenic irradiation for patients unfit for splenectomy 2
  3. Splenectomy in selected cases with hypersplenism or massive symptomatic splenomegaly 3, 4

• Tumor flare reactions during lenalidomide treatment for CLL may be accompanied by spleen enlargement along with painful lymph nodes, low-grade fever, rash, and/or bone pain 1

Indications for Splenectomy

• Splenectomy may be considered in CLL patients with:

  • Massive symptomatic splenomegaly causing significant discomfort
  • Hypersplenism leading to cytopenias (anemia, thrombocytopenia)
  • Failure to respond to medical therapy 4

• However, the benefit of splenectomy must be weighed against surgical risks and the patient's life expectancy 3

Monitoring

• Spleen size should be regularly assessed during follow-up of CLL patients
• Progressive splenomegaly may indicate disease progression requiring treatment modification
• In clinical trials, response assessment includes evaluation of splenomegaly, with reduction in spleen size being part of response criteria 1

Cautions and Considerations

• The presence of splenomegaly in CLL does not automatically necessitate treatment unless it causes symptoms or is associated with progressive disease
• Splenectomy should be approached with caution in CLL patients, as benefits may be limited by disease stage and potential for recurrent cytopenias 3
• Post-splenectomy, patients require appropriate vaccinations and oncologic follow-up 5