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					Chronic Leukemia
 Kristine Krafts, M.D.
 November 10, 2009
Hematopoietic Malignancies

        Leukemias
    • Acute leukemias
    • Chronic leukemias

       Lymphomas
   • Hodgkin lymphoma
   • Non-Hodgkin lymphoma

   Plasma cell disorders
     • Multiple myeloma
Hematopoietic Malignancies

         Leukemias
    • Acute leukemias
       • Acute myeloid leukemia
       • Acute lymphoblastic leukemia
    • Chronic leukemias
Chronic myeloid leukemia   Chronic lymphocytic leukemia
Hematopoietic Malignancies

        Leukemias
    • Acute leukemias
    • Chronic leukemias
       • Chronic myeloproliferative disorders
       • Chronic lymphoproliferative disorders
Hematopoietic Malignancies

        Leukemias
    • Acute leukemias
    • Chronic leukemias
       • Chronic myeloproliferative disorders
Chronic Myeloproliferative Disorders


 • Malignant proliferation of myeloid cells
   (not blasts, but maturing cells) in blood,
   bone marrow
 • Four disorders: CML, MF, PV, ET
 • Occur in adults
 • Long course
Chronic Myeloproliferative Disorders

             Four disorders

     • Chronic myeloid leukemia
     • Polycythemia vera
     • Essential thrombocythemia
     • Chronic myelofibrosis
Chronic Myeloproliferative Disorders

   Features common to all four disorders:

       • Occur only in adults
       • Long clinical course
       • ↑ WBC with left shift
       • Hypercellular marrow
       • Big spleen
Chronic Myeloid Leukemia (CML)

       Things you must know

   • Neutrophilic leukocytosis
   • Basophilia
   • Philadelphia chromosome
   • Three phases
Chronic Myeloid Leukemia
Chronic Myeloid Leukemia
   Normal     Chromosomes
chromosomes      in CML
Cytogenetics
Chronic Myeloid Leukemia

       Three phases

    • Chronic phase
       • Stable counts
       • easily controlled
       • 3-4 years
    • Accelerated phase
       • unstable counts
       • fatal within months
    • Blast crisis
       • lots of blasts
       • fatal within weeks
  Chronic Myeloid Leukemia


• Treatment
  • Used to be bone marrow transplant
  • Now, imatinib!

• Prognosis
  • Median survival used to be 5-6 years
  • Now, ????
Chronic Myelofibrosis (MF)

    Things you must know


 • Panmyelosis…
 • …then marrow fibrosis
 • Extramedullary hematopoiesis
Chronic Myelofibrosis
Chronic Myelofibrosis
     Polycythemia Vera (PV)

        Things you must know


• High RBC in blood (makes blood sludgy)
• Different from secondary polycythemia!
Polycythemia vera
Essential Thrombocythemia (ET)

         Things you must know


• Very high platelet count in blood
• Different from secondary thrombocythemia!
Essential thrombocythemia
Hematopoietic Malignancies

        Leukemias
    • Acute leukemias
    • Chronic leukemias
       • Chronic myeloproliferative disorders
       • Chronic lymphoproliferative disorders
Chronic Lymphoproliferative Disorders


  • Malignant proliferation of lymphoid cells
    (not blasts, but maturing cells) in blood,
    bone marrow
  • Many disorders; CLL most important
  • Occur only in adults
  • Long course; indolent but incurable
Chronic Lymphocytic Leukemia (CLL)

           Things you must know

  • Malignant proliferation of small, mature-
    appearing lymphoid cells
  • Same thing as small lymphocytic
    lymphoma!
  • Funny phenotype: B cells, but also CD5+
  • Long but inexorable clinical course; death
    from infection
Chronic lymphocytic leukemia
Chronic lymphocytic leukemia
Chronic Lymphocytic Leukemia


• Treatment
  • Conservative!
  • Corticosteroids, chemotherapy only
    when symptomatic

• Prognosis
  • Depends on “counts” and extent of
    involvement of marrow and nodes
  • Mean survival 13-15 years
  • Death usually results from infection

				
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posted:3/29/2010
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