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Comprehensive Insights into the Immunophenotype and Scoring Systems in CLL Management

This overview encapsulates critical findings on the immunophenotype of Chronic Lymphocytic Leukemia (CLL), including scoring systems based on various markers like SmIg, CD5, CD23, and FMC7. We analyze CLL disease scores, the role of bone marrow trephine biopsies in prognosis, and chromosomal abnormalities associated with disease progression. Additionally, a summary of genetic factors and treatment agents such as chlorambucil and rituximab provides a holistic view of CLL management.

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Comprehensive Insights into the Immunophenotype and Scoring Systems in CLL Management

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  1. Table 1:The Immunophenotype of CLL Tests used as basis for a scoring system Marker (result) Score SmIg (weak) 1 CD5 (+) 1 CD23 (+) 1 FMC7 (-)* 1 CD79b (- or -/+) 1 Total 5 *Epitope of CD20 but CD20 not useful for scoring

  2. Table 2: CLL SCORE IN B-CELL DISORDERS Disease Score CLL typical 4-5 atypical; CLL/PL 3-5 B-Prolymphocytic Leukaemia 0-1 Hairy Cell Lukaemia 0-1 NHL with leukaemia*0-2 * Follicular, Mantle, Splenic Marginal Zone

  3. Table 3: Causes of Anaemia in CLL and their management

  4. TABLE 4: THE VALUE OF BONE MARROW TREPHINE BIOPSIES IN CLL • Prognostic Feature • Diffuse = packed BM has poor prognosis • Clarify the nature of Cytopenias • Low Platelets > megakaryocytes • Red cell aplasia • Differential diagnosis with low grade NHL • Paratrabecular pattern not seen in CLL; • > Proliferation centres in CLL/PL • To assess response to treatment • Nodular PR only seen in biopsy

  5. TABLE 5: Hierarchical Model of Chromosomal Abnormalities in CLL Karyotype Döhner CLL4 No of patients 352 223 17p deletion 7% 7% 11q deletion 17% 17% 12q trisomy 14% 10% Normal karyotype 18% 26% 13q del as sole abnormality 36% 35% Other abnormalities 8% 5%* * 6q21 deletion

  6. Table 6:Genetic abnormalities associated with progression and poor prognosis in CLL • Younger age • Lymphadenopathy • Short survival 11q23 deletions (20% of cases) • CLL/PL • Disease progression • High proliferation rate Trisomy 12 (15% of cases) • CLL/PL • Poor response to therapy • Transformation Abnormal 17p21 (p53 mutations/deletions 7% of cases)

  7. Table 7: MUTATIONAL STATUS OF Ig VH GENES IN CLL Unmutated Mutated Incidence 45% 55% M:F ratio 11:1 1.1:1 Morphology atypical typical Trisomy 12 frequent infrequent Abnormal 13q14 rare common Stage (BiNet) B&C (2/3) stage A (2/3) Disease course progressive stable CD38/ZAP70 expressed negative Therapy required not needed Response poor normal Survival short long

  8. Table 8: AGENTS USED ALONE OR IN COMBINATION FOR THE TREATMENT OF CLL • Chlorambucil (oral) • Cyclophosphamide (oral/IV) • Prednisolone (standard/high dose) • Fludarabine (oral/IV) • Cladribine (2-CdA; IV/SC) • Pentostatin (DCF; IV) • Rituximab (anti-CD20) • Campath-1H (anti-CD52; IV/SC) • Genasense (BCL2 antisense) • Zevalin (Rituximab + Yttrium 90)

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