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Snapshot
  • A 62-year-old Caucasian man complains of months of fatigue. He notices that his skin seems abnormally yellow. On physical exam, he has notable tender lymphadenopathy. Peripheral smear reveals some spherocytes and lymphocytosis. His leukocyte count is 35,000/μL with 95% lymphocytes and a Hb of 7 g/dL. His direct Coombs test is positive with anti-IgG. He is started on fludarabine.
Introduction

  • Clonal proliferation of incompetent B-cells
    • small lymphocytic lymphoma (SLL)
      • disease is mainly in lymph nodes
    • chronic lymphocytic lymphoma (CLL)
      • disease in blood and bone marrow
  • Epidemiology
    • > 60 years of age
    • most common form of leukemia in adults in Western countries
    • more common in Caucasian males
  • Many are diagnosed incidentally with markedly elevated WBC
  • Insidious onset of symptoms
  • Associated conditions
    • warm autoimmune hemolytic anemia (IgG)

 

Presentation
  • Symptoms
    • many are asymptomatic at presentation
    • most common symptom is fatigue
    • recurring infections from incompetent lymphocytes
      • pneumonia
      • zoster
    • early satiety
    • mucocutaneous bleeding from thrombocytopenia
  • Physical exam
    • painful lymphadenopathy
    • hepatosplenomegaly
Evaluation
  • Labs – CBC
    • ↑ WBC (> 20,000/μL) with > 80% lymphocytes
    • ↓ RBCs (attacked by CLL cells)
    • ↓ PLTs (attacked by CLL cells)
  • ↓ IgG in half of patients
  • Most accurate test
    • flow cytometry – CD5, CD19, CD20, and CD23+ B-cells
  • Peripheral blood smear
    • smudge cells = hallmark
      • artifact of lab, from cover slip crushing the nucleus
Differential Diagnosis
  • AIHA
  • Mantle cell lymphoma
  • ALL
  • Hairy cell lymphoma
Treatment
  • Treatment only for symptomatic or rapid progression
    • fludarabine – first-line
    • fludarabine plus rituximab for symptomatic patients
  • Curative therapy
    • allogeneic stem cell transplant
Prognosis, Prevention, and Complications
  • Prognosis
    • most live 5-10 years after diagnosis
    • some die rapidly, within 2-3 years
  • Complications
    • Richter syndrome/transformation
      • transformation of CLL into aggressive large B-cell lymphoma
      • 3-10% of cases
      • weight loss, fevers, night sweats, cachexia, and lymphadenopathy
      • poor prognosis
    • infections secondary to hypogammaglobulinemia
 

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