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Table 18.1 Classification of the chronic 
lymphoid leukaemias. 
B-cell T-cell 
Chronic lymphocytic 
leukaemia (CLL) 
Prolymphocytic 
leukaemia (PLL) 
Hairy cell leukaemia 
(HCL) 
Plasma cell leukaemia 
Large granular 
lymphocytic 
leukaemia 
T-cell prolymphocytic 
leukaemia (T-PLL) 
Adult T-cell leukaemia/ 
lymphoma 
Sézary syndrome (see 
Chapter 20) 
Source: WHO (2008) classification (see p. 426). 
Table 18.2 Immunophenotype of the chronic B-cell leukaemias/lymphomas (all cases CD19+). 
CLL PLL HCL FL MCL 
SIg Weak ++ ++ ++ + 
CD5 + − − − + 
CD22/FMC7 − + + + ++ 
CD23 + − − − − 
CD79b − ++ −/+ ++ ++ 
CD103* −/+ − + − − 
CLL, chronic lymphocytic leukaemia; FL, follicular lymphoma; HCL, hairy cell leukaemia; MCL, mantle cell lymphoma; PLL, 
prolymphocytic leukaemia. 
* CD103 is positive only in HCL. 
Table 18.3 Prognostic factors in chronic lymphocytic leukaemia. 
Good Bad 
Stage Binet A (Rai 0–I) Binet B, C (Rai II–IV) 
Sex Female Male 
Lymphocyte doubling time Slow Rapid 
Bone marrow biopsy appearance Nodular Diffuse 
Chromosomes Deletion 13q14 Deletion 17p 
VH immunoglobulin genes Hypermutated Unmutated 
Use of VH3.21 
ZAP expression Low High 
CD38 expression Negative Positive 
LDH Normal Raised 
LDH, lactate dehydrogenase.
Table 18.4 Staging of chronic lymphocytic leukaemia (CLL). 
(a) Rai classification 
Stage 
0 Absolute lymphocytosis >15 × 109/L+ 
I As stage 0 + enlarged lymph nodes (adenopathy) 
II As stage 0 + enlarged liver and/or spleen ± adenopathy 
III As stage 0 + anaemia (Hb <10.0 g/dL)+ ± adenopathy ± organomegaly 
IV As stage 0 + thrombocytopenia (platelets <100 × 109/L)+ ± adenopathy ± organomegaly 
(b) International Working Party classification 
Stage Organ enlargement* Haemoglobin† (g/dL) Platelets† (× 109/L) 
A (50–60%) 0, 1 or 2 areas 
B (30%) 3, 4 or 5 areas ≥10 ≥100 
C (<20%) Not considered <10 and/or <100 
* One area = lymph nodes >1 cm in neck, axillae, groins or spleen, or liver enlargement. 
† Secondary causes of anaemia (e.g. iron deficiency) or autoimmune haemolytic anaemia or autoimmune thrombocytopenia 
must be treated before staging. 
Source: (b) Binet J.L. et al. (1981) Cancer 48, 198.

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Chapter18

  • 1. Table 18.1 Classification of the chronic lymphoid leukaemias. B-cell T-cell Chronic lymphocytic leukaemia (CLL) Prolymphocytic leukaemia (PLL) Hairy cell leukaemia (HCL) Plasma cell leukaemia Large granular lymphocytic leukaemia T-cell prolymphocytic leukaemia (T-PLL) Adult T-cell leukaemia/ lymphoma Sézary syndrome (see Chapter 20) Source: WHO (2008) classification (see p. 426). Table 18.2 Immunophenotype of the chronic B-cell leukaemias/lymphomas (all cases CD19+). CLL PLL HCL FL MCL SIg Weak ++ ++ ++ + CD5 + − − − + CD22/FMC7 − + + + ++ CD23 + − − − − CD79b − ++ −/+ ++ ++ CD103* −/+ − + − − CLL, chronic lymphocytic leukaemia; FL, follicular lymphoma; HCL, hairy cell leukaemia; MCL, mantle cell lymphoma; PLL, prolymphocytic leukaemia. * CD103 is positive only in HCL. Table 18.3 Prognostic factors in chronic lymphocytic leukaemia. Good Bad Stage Binet A (Rai 0–I) Binet B, C (Rai II–IV) Sex Female Male Lymphocyte doubling time Slow Rapid Bone marrow biopsy appearance Nodular Diffuse Chromosomes Deletion 13q14 Deletion 17p VH immunoglobulin genes Hypermutated Unmutated Use of VH3.21 ZAP expression Low High CD38 expression Negative Positive LDH Normal Raised LDH, lactate dehydrogenase.
  • 2. Table 18.4 Staging of chronic lymphocytic leukaemia (CLL). (a) Rai classification Stage 0 Absolute lymphocytosis >15 × 109/L+ I As stage 0 + enlarged lymph nodes (adenopathy) II As stage 0 + enlarged liver and/or spleen ± adenopathy III As stage 0 + anaemia (Hb <10.0 g/dL)+ ± adenopathy ± organomegaly IV As stage 0 + thrombocytopenia (platelets <100 × 109/L)+ ± adenopathy ± organomegaly (b) International Working Party classification Stage Organ enlargement* Haemoglobin† (g/dL) Platelets† (× 109/L) A (50–60%) 0, 1 or 2 areas B (30%) 3, 4 or 5 areas ≥10 ≥100 C (<20%) Not considered <10 and/or <100 * One area = lymph nodes >1 cm in neck, axillae, groins or spleen, or liver enlargement. † Secondary causes of anaemia (e.g. iron deficiency) or autoimmune haemolytic anaemia or autoimmune thrombocytopenia must be treated before staging. Source: (b) Binet J.L. et al. (1981) Cancer 48, 198.