Chronic Lymphocytic Leukemia - Summary
Cancer affecting lymphocytes/Mature B cell neoplasm
• Lymphocytes accumulate in large numbers and are dysfunctional rendering the immune system incompetent
• CLL is identical to the non-Hodgkin lymphoma SLL
CLL Epidemiology:
• Most common leukemia in adults in the west
• M > F
• Affects mainly older adults
• Exposure to Agent Orange or Hep C
Clinical Presentation:
• Many with no early symptoms
• Lymphadenopathy
• Fevers > 100.5 for > 2 weeks without infection
• Drenching night sweats
• Extreme fatigue
Physical Exam/Signs:
• Lymphadenopathy
• Splenomegaly
• Hepatomegaly
• Skin:
- CLL cells can infiltrate any organ.
- Leukemia cutis involves face: macules, papules, plaques, nodules, ulcers, blisters.
• MPGN: Paraneoplastic phenomenon
CLL Diagnosis:
• Lymphocytosis on CBC
• Confirm: Flow cytometry
• Peripheral smear: Smudge cells or basket cells can indicate disease
• B cell surface molecules: CD5 and CD23 antigen
• Matute’s CLL Scoring system: DDX between classical CLL and other C Cell lymphoproliferative disorders.
CLL Labs:
1. Lymphocytosis: Absolute blood lymphocyte > 5000/microL
2. Cytopenias:
• Neutropenia, anemia, thrombocytopenia
• AIHA
• Pure red cell aplasia
• ITP
• Agranulocytosis
3. Immunoglobulin abnormalities:
• Hypogammaglobulinemia
• Polyclonal increase in gamma globulins
4. Elevated LDH
5. Elevated beta 2 microglobulin
Treatment:
• Bruton's tyrosine kinase (BTK)
Complications:
• Hypogammaglobulinemia
• Warm AIHA
• Bone marrow failure
• Richter’s Syndrome: Transform to DLBCL, PML leukemia, Hodgkin’s lymphoma or acute leukemia
• GI complications: Intussusception, SIB Contamination, colitis
DDX:
• Follicular lymphoma
• Marginal zone lymphoma
• Mantle cell lymphoma
• Lymphoplasmacytic lymphoma
• Hairy cell leukemia
• Prolymphocytic leukemia
• Sezary syndrome
• Smoldering adult T cell leukemia
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