B Cell Chronic Lymphoid Leukemia / Small Lymphocytic Lymphoma

  • Most common lymphoid leukemia
  • 7% of all NHL when presenting as lymphoma
  • Rare in children

Clinical Presentation

  • B symptoms in 1/3
  • Typically found incidentally
  • Lymphoma presentation
    • Asymptomatic lymphadenopathy
    • +/- splenomegaly
  • Autoimmune complications are not uncommon
    • 50/50 split between normal Ig genes and mutated Ig genes
    • AIHA
    • AI thrombocytopaenia
    • Hypogammaglobulinaemia
    • Red cell aplasia

Diagnosis

  • Increased number of monoclonal CD5+ B cells in peripheral blood of FNA if presenting as lymphoma
    • Even when presenting as lymphoma 75% will have BM involvement
  • Bone marrow infiltration clinches the diagnosis
  • Peripheral smear shows
    • Smudge / basket cells (nuclear remnants of cells damaged by shear stress of making smear)
  • Cytogenetics
    • Trisomy 12 in 30%
    • Chromosome 13 abnormalities

Treatment

  • Watchful waiting
    • If only BM involvement and lymphocytosis
    • If adequate circulating blood counts, asymptomatic despite lymphadenopathy +/- splenenomegaly
  • Manage AI complicaitons independent of treating CLL
  • FCR (fludarabine/cyclophosphamide/rituximab) for well patients
  • Chlorambucil more gentle but fludarabine has better outcomes – chlorambucil can be given in elderly patients
  • Bendamustine also highly effective
  • RCHOP can be used in lymphoma presentation
  • Allogenic BM transplant can be curative but has major mortality

Prognosis

  • Patients with unmutated Ig genes tend to have worse outcomes
    • ZAP 70 cirrekateds with unmutated Ig genes but not validated yet
    • CD38 anecdotally is low in mutated Ig genes and high in unmutated genes but not validated yet
  • 50% 5-year survival
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