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Perth Haematology :: Dr Steven Ward

CLL

 

Chronic Lymphocytic Leukaemia

 

CLL is an indolent disorder.

 

 

Staging

Rai System

 

Binet System

Stage

Description

Risk

 

Stage

Description

0

Lymphocytosis >15.0 and >40% BM

Good

 

A

Hb ≥100g/L, Plat ≥ 100 <3 enlarged areas

I

Stage 0 with enlarged node(s)

Intermed

 

B

Hb≥100g/L, Plat≥100

≥3 enlarged areas

II

+ Splenomegaly, hepatomegaly or both

Intermed

 

C

Hb  < 100, or plat <100 with any enlarged areas

III

Hb <110 g/L Hct<33%

High

 

 

 

IV

Platelets <100

High

 

 

 

Immune-mediated cytopenia is not the basis for staging

 

 

CLL Management

Often no treatment is needed; or treatment is not required until the disease progresses, which can take many years. Many patients live with the disorder for many years.

 

Indications for treatment are:

bullet

Auto-immune disorder: ITP, AIHA, PRCA

bullet

Recurrent infections

bullet

Marked B-symptoms: weight loss, fevers, night sweats

bullet

End-organ impairment due to nodes

bullet

Marrow failure: cytopenia

bullet

Bulky disease: many large nodes; very high lymphocyte count

bullet

Steady progression: rapid doubling time of cells (< 6 months)

bullet

Histological transformation (uncommon)

 

Treatment Options:

bullet

Radiotherapy may be used for localised disease

bullet

Chlorambucil ± Prednisolone ± Rituximab

bullet

Cyclophosphamide ± Prednisolone ± Rituximab

bullet

CVP (Cyclophosphamide, Vincrsitine, Prednisolone) ± Rituximab

bullet

FC (Fludarabine, cyclophosphamide) ± Rituximab

bullet

Alemtuzumab (CamPath)

 

bullet

IVIg for hypogammaglobulinaemia with recurrent sepsis

 

 

 

CLL Response Criteria

 

 

CR

PR
PD

Exam

Normal

≥ 50% decrease

≥ 50% increase or new

Symptoms

None

 

 

Lymphocytes

≤4.0

≥50% decrease from baseline

>50% increase

Neutrophils

≥1.5

≥1.5 or 50% improvement over baseline

 

Platelets

>100

>100 or 50% improvement over baseline

 

Hb

>110

>110 or 50% improvement over baseline

 

BM lymphocytes

<30% ; no nodules

 

 

Other

Duration ≥2 months

Duration ≥ 2 motnhs

Richter’s

 

CLL Prognostic Markers

 

Data

Favourable

Adverse

Rai / Binet Stage

0,1   A

2,3,4    B,C

Lymphocyte doubling time

> 12 months

< 12 months

BM histology

Nodular, interstitial, mixed

Diffuse

% prolymphocytes

<10%

10-55%

Gender, age

Female

Male, elderly

PS

Normal

Poor

Response to therapy

Good

Poor

Serological markers

 

β2M, sCD23, TK, LDH

Phenotype

sCD38-, sCD62L/54/49c/25+

cZAP70-

nKi-67-

sCD38+

cZAP70+

nKi-67+

Karyotype / FISH

Normal, 13q-

12+, 11q-, 17p-. Complex

IgVH family

 

V1-69, V3-21

IgV gene

Mutated

Unmutated

Telomere

Normal

Short