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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. Table 2: CLL SCORE IN B-CELL DISORDERS. Disease Score
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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
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
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
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
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)
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
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)