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    Cll

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    Aswini Sugumaran

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    © All Rights Reserved
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    Cll

    Uploaded by

    Aswini Sugumaran
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    0 views19 pages

    Cll

    Uploaded by

    Aswini Sugumaran

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    © All Rights Reserved
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    Chronic Lymphocytic Leukemia

    Definition
    • Clonal B cell malignancy.
    • Progressive accumulation of long lived
    mature lymphocytes.
    • Increase in anti-apoptotic protein bcl-2.
    • Intermediate stage between pre-B and
    mature B-cell.
    Epidemiology
    • Most common leukemia of Western world.
    • Less frequent in Asia and Latin America.
    • Male to female ratio is 2:1.
    • Median age at diagnosis is 65-70 years.
    • In US population incidence is similar in
    different races.

    Cancer statastics 2000; CA J Clin


    2000; 50:7-33
    Etiology & Risk factors
    • High familial risk with two-fold to seven-
    fold higher risk.
    • No documented association with
    environmental factors.
    • No established viral etiology.
    Diagnostic Criteria
    • Defined by NCI & IWCLL.
    • Persistent lymphocytosis.
    • Absolute count more than 5000.
    • Mature appearing B-cells with <10% of
    prolymhocytes

    Blood 1996; 87: 4990


    Flow Cytometry
    • CD 19 and/or CD 20 are always co
    expressed with CD 5.
    • Weak expression of surface
    immunoglobulins (sIgM & sIgD).
    • CD 21, CD 23, CD 24 may be expressed.
    Bone Marrow
    • Not required for diagnosis.
    • Recommended to estimate the extent for
    prognostic implications.
    • Diffuse infiltration has poor prognosis.
    Cytogenetics
    • Deletions in chromosome 13 at q 14
    • Chromosome 11 at q22 or q23.
    • Trisomy-12.
    • Less common are deletions in chromosome
    17 & 6.

    J Mol Med 1999; 77:266


    Clinical Features
    • Disease of elderly with wide spectrum of
    clinical features.
    • 20% are asymptomatic.
    • Classic B symptoms.
    • Variable physical findings with normal to
    diffuse LAD , hepato/splenomegaly.
    Other Labs
    • Hypogammaglobulinemia seen >50%.
    • 5-10% have small monoclonal peak.
    • Positive Coombs’ test in 30% .
    • Autoimmune hemolytic anemia &
    thrombocytopenia in <10%
    The Rai Staging System
    Stage 0 Lymphocytosis only (> 15,000/mm3)
    Stage 1 Lymphocytosis and lymphadenopathy
    Stage 2 Lymphocytosis and splenomegaly with
    or without lymphadenopathy
    Stage 3 Lymphocytosis and anemia (Hgb <11 g/dL)
    with or without lymphadenopathy or
    hepatosplenomegaly
    Stage 4 Lymphocytosis and thrombocytopenia (Plt <
    100,000/UL) with or without anemia,
    lymphadenopathy or hepatosplenomegaly
    Modified Rai Staging g.
    • Low-risk: stage 0, MS > 13 years.
    • Intermediate-risk: stage I & II with MS
    about 8 years.
    • High-risk: stages III & IV with MS about

    3 years
    The Binet Staging System
    Stage A No anemia, no thrombocytopenia,
    <3 involved nodal areas
    Stage B No anemia, no thrombocytopenia,
    >=3 involved nodal areas
    Stage C Anemia (Hgb < 10 g/dL) and/or
    thrombocytopenia (Plt <
    100,000/uL)
    Other Prognostic Features
    • Bone marrow pattern of lymphoid infiltration.
    • Lymphocyte doubling time.
    • Serum beta-2-microglobulin.
    • Mutational status of Ig V and CD 38
    expression have recently been identified.

    Blood 94: 1848-1854, 1999


    Treatment
    • Nucleoside analogs like Fludarabine is the
    drug of choice.
    • More effective in higher CR & longer PFS
    compared to alkyalating agents.
    • No survival advantage.
    • Fludarabine in various combinations with
    Cytoxan & Rituxan are widely used.

    Blood 1996; 88 (suppl 1): 141a


    Monoclonal antibodies
    • Campath (anti-CD 52) is recently approved
    for refractory CLL.
    • Results in profound lympocytopenia, both
    B and T cells are destroyed.
    • Rituxan (anti CD 20) is widely used both as
    single agent and in combination.

    Blood 1999; 94 (suppl 1); 705a


    Bone marrow transplantation
    • Allogenic BMT is a viable option in
    younger patients.
    • Durable response rates seen in advanced,
    refractory disease.
    • Autologous BMT using purged marrow
    have been investigated in elderly.

    J Clin Oncol 16:2817-2724


    Transformation
    • Large- cell lymphoma/ Richter’s
    – Aggressive presentation
    – Extranodal involment
    – Sharp rise in LDH
    – CHOP is standard treatment.
    • Prolymphoctic leukemia.
    – > 55% increase in prolymphocytes
    – Progression of splenomegaly & cytopenias
    – Refractoriness to treatment.

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