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    Chronic Lymphocytic Leukemia (CLL)

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    Vikkineshwaran Siva Subramaniam
    Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults. It is a cancer of B-cell lymphocytes that accumulate in the blood and bone marrow over time. CLL usually progresses slowly and may not require treatment for many years. Prognosis is based on staging systems like Rai or Binet that classify CLL based on symptoms and organ involvement. Factors like genetic abnormalities, lymphocyte doubling time, and response to treatment can also provide information on prognosis and guide treatment decisions, which typically involves chemotherapy, immunotherapy, or watchful waiting.

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    Chronic Lymphocytic Leukemia (CLL)

    Uploaded by

    Vikkineshwaran Siva Subramaniam
    655 views38 pages
    Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults. It is a cancer of B-cell lymphocytes that accumulate in the blood and bone marrow over time. CLL usually progresses slowly and may not require treatment for many years. Prognosis is based on staging systems like Rai or Binet that classify CLL based on symptoms and organ involvement. Factors like genetic abnormalities, lymphocyte doubling time, and response to treatment can also provide information on prognosis and guide treatment decisions, which typically involves chemotherapy, immunotherapy, or watchful waiting.

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    Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults. It is a cancer of B-cell lymphocytes that accumulate in the blood and bone marrow over time. CLL usually progresses slowly and may not require treatment for many years. Prognosis is based on staging systems like Rai or Binet that classify CLL based on symptoms and organ involvement. Factors like genetic abnormalities, lymphocyte doubling time, and response to treatment can also provide information on prognosis and guide treatment decisions, which typically involves chemotherapy, immunotherapy, or watchful waiting.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    655 views38 pages

    Chronic Lymphocytic Leukemia (CLL)

    Uploaded by

    Vikkineshwaran Siva Subramaniam
    Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults. It is a cancer of B-cell lymphocytes that accumulate in the blood and bone marrow over time. CLL usually progresses slowly and may not require treatment for many years. Prognosis is based on staging systems like Rai or Binet that classify CLL based on symptoms and organ involvement. Factors like genetic abnormalities, lymphocyte doubling time, and response to treatment can also provide information on prognosis and guide treatment decisions, which typically involves chemotherapy, immunotherapy, or watchful waiting.

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    At a glance
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    Some key takeaways are that CLL is the most common leukemia in adults, affects B-cells in most cases, and usually has an indolent clinical course. It often presents with lymphocytosis and lymphadenopathy and is diagnosed based on lymphocyte count and phenotype analysis.

    Some main clinical features of CLL include asymptomatic lymphocytosis, lymphadenopathy, hepatosplenomegaly, anemia, thrombocytopenia, fatigue, weight loss and frequent infections. However, approximately 70% of patients are asymptomatic at diagnosis.

    The main diagnostic criteria for CLL are: 1) a peripheral blood lymphocyte count greater than 5 G/L with less than 55% atypical cells, 2) presence of B-cell antigens CD19, CD20 and CD24 with CD5 positivity, 3) greater than 30% lymphocytes on bone marrow aspirate.

    Chronic Lymphocytic Leukemia

    Definition Etiology Pathology Clinical Findings Laboratory Findings Diagnostic Criteria Differential Diagnosis Investigations Staging Prognosis Treatment
    Vikkineshwaran
    1

    Chronic Lymphocytic Leukemia (CLL)


    CLL is the most common leukemia in the western world. It is an adult leukemia that affects all organs. Disease of the elderly
    2

    Accumulation of nonproliferating mature-appearing lymphocytes in the blood, marrow, lymph nodes, and spleen
    ~99% cases: B cell related ~1% cases: T cell related
    3

    It is slowly progressive, with a predictable clinical course and survival of 25 years and often does not require therapy. Considerably less aggressive disorder than are the other leukemias.

    Etiology
    The cause of CLL is unknown There is increased incidence in farmers, rubber manufacturing workers, asbestos workers, and tire repair workers Genetic factors have been postulated to play a role in high incidence of CLL in some families

    Etiology
    Cytogenetics
    clonal chromosomal abnormalities are detected in approximately 50% of CLL patients the most common clonal abnormalities are:
    trisomy 12 structural abnormalities of chromosomes 13, 14 and 11

    patients with abnormal karyotypes have a worse prognosis


    6

    B-CELL CHRONIC LYMPHOCYTIC LEUKEMIA TRISOMY FOR

    CHRONIC LYMPHOCYTIC LEUKEMIA 13q-CHROMOSOME ABNORMALITY

    CLL - Pathology
    Genetic change in B-cell clone Proliferation exceeds apoptosis Gradual accumulation of neoplastic B-lymphocytes blood, marrow, nodes, spleen

    Normal adult peripheral blood:


    B-cell lymphocytes 30% with surface immunoglobulins T-cell lymphocytes 70% without surface immunoglobulins
    10

    Very important to differentiate which type of lymphocytes are involved in the CLL (B cells or T cells) Diagnosis of CLL can be done morphologically, but distinguishing between T-cell and B-cell CLL requires testing for cluster differentiation (CD) antigens
    11

    Malignant B cells of CLL do not progress to the final stages of development, the plasma cells;
    Appear to stop developmentally at earlier Blymphocyte stage of development

    CLL usually presents with elevated WBC count with many lymphocytes (lymphocytosis)
    12

    Common Sx:
    Fever Pallor Fatigue Shortness of breath Easy bruising Gingival bleeding Weight loss Frequent infections

    Note: approximately 70% of patients are asymptomatic at the time of diagnosis


    13

    CLL clinical features


    Asymptomatic lymphocytosis Marrow failure Lymphadenopathy Hepatosplenomegaly B-symptoms Immunodeficiency Anemia/Thrombocytopenia
    14

    B symptoms
    Systemic symptoms of
    fever, night sweats, and weight loss

    associated with both Hodgkin's lymphoma and non-Hodgkin's lymphoma. The presence or absence of B symptoms has prognostic significance and is reflected in the staging of these lymphomas.
    15

    Changes in other organs


    The lymph nodes, liver and spleen are characteristically involved. In nodes and spleen the normal architecture becomes completely effaced by the infiltrate of monomorphic small lymphocytes, and similar cells are present in the portal tracts of the liver.
    16

    Production of non-functional Ig, so there is a hypogammaglobulinemia. Other blood elements are also reduced, which is what usually brings patients in. There may be
    anemia's, thrombocytopenia's, or neutropenia's.

    17

    Small Cell Lymphocytic Lymphoma


    Sometimes, the predominant presentation is with lymph node involvement with little or no evidence of disease in the blood and marrow, when it is termed small cell lymphocytic lymphoma(SCLL).

    18

    Lymph node biopsy

    The cells are round to oval and lack significant nuclear angularity or cleavage. The chromatin is coarse and clumped. benign mature small lymphocytes also have same characteristics It may be hard to tell them apart
    19

    Laboratory and Radiographic Work-up:


    CBC with manual differential Peripheral smear Flow cytometry Chemistry studies to check for organ dysfunction Lymph node biopsy

    20

    Normal
    lymphocytes

    21

    These mature lymphocytes are increased markedly in number.

    22

    The CLL cells tend to fragment during preparation of the blood film, producing many smudge cells'

    23

    Diagnosis Peripheral smear

    Small, mature looking Lymphocytes

    24

    Diagnosis Flow Cytometry

    Co-expression of CD5 and CD19,23

    25

    Diagnostic criteria
    1) A peripheral blood lymphocyte count of greater than 5 G/L, with less than 55% of the cells being atypical 2) The cell should have the presence of B cell-specific differentiation antigens (CD19, CD20, and CD24) and be CD5(+) 3) A bone marrow aspirates showing greater than 30% lymphocytes

    26

    Differential diagnosis
    Infectious causes
    bacterial (tuberculosis) viral (mononucleosis)

    Malignant causes
    B-cell T-cell
    leukemic phase of non-Hodgkin lymphomas Hairy-cell leukemia Waldenstrom macroglobulinemia large granular lymphocytic leukemia
    27

    Staging
    RAI Binet

    28

    Staging Rai
    Stage Feature 0 1 2 3 4 Lymphocytosis only Lymphadenopathy Median Survival >150 months 101

    Spleno/Hepato megaly 71 Anemia Thrombocytopenia 19 19


    29

    Staging - Binet
    Stage Features A Survival

    Lymphocytosis and <3 areas of > 7 yrs Lymphadenopathy

    B C

    Lymphocytosis and >3 areas of < 5 yrs Lymphadenopathy Anemia (<10) or Thrombocytopenia (<100) <2 yrs

    30

    The clinical course and staging of chronic lymphocytic leukemia. Five stages are recognized in the 'Rai' classification.

    31

    Prognostic Factors:
    Prognosis is based on RAI classification Treatment based on Binet classification
    32

    Markers of poor prognosis


    Advanced Rai or Binet stage Peripheral lymphocyte doubling time <12 months Diffuse marrow histology Increased number of prolymphocytes or cleaved cells Poor response to chemotherapy High 2- microglobulin level
    Abnormal karyotyping
    33

    CLL - median survival (years)


    Early - lymphocytosis alone (>10y) Late - marrow failure (3-4y)

    34

    Prognostic Variables: Cytogenetics


    13q deletion 12q trisomy, Normal 11q deletion

    17q deletion

    35
    Dohner H et al. N Engl J Med 2000;343:1910-1916

    CLL - Complications
    Immune Deficiency: Recurrent infections, hypo-gammaglobulinemia, increased risk of secondary malignancies Immune dysregulation: Autoimmune Phenomena (ITP, AIHA) Richters Transformation: the leukemia changes into a fast-growing diffuse large B cell lymphoma Opportunistic infection (e.g. shingles, pneumonia)
    36

    CLL - principles of treatment


    Watch and Wait
    Not usually curable with available therapy Treatments include
    bone marrow transplants, radiation, chemotherapy, and intravenous gamma globulin to help prevent bacterial infections
    37

    FYI: Umbilical cords look like smiley faces!

    38

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