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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