Page 254 - Essential Haematology

240 / Chapter 18 Chronic lymphoid leukaemias

Table 18.4 Staging of chronic lymphocytic leukaemia ( CLL ).

(a) Rai classifi cation
Stage
9
+
0 Absolute lymphocytosis > 15 × 10 /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

+
9

IV As stage 0 + thrombocytopenia (platelets < 100 × 10 /L) ± adenopathy ± organomegaly

(b) International Working Party classifi cation
†
9
†

Stage Organ enlargement * Haemoglobin (g/dL) Platelets ( × 10 /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 defi ciency) or autoimmune haemolytic anaemia or autoimmune thrombocytopenia
must be treated before staging.
Source : (b) Binet J.L. et al . (1981) Cancer 48 , 198.

not effective in patients who are resistant to R - FC. haemoglobin levels. The peripheral lymphocyte

Combination therapy with rituximab is being count initially rises as infiltrated organs shrink, but
explored. later the count falls. High dose steroid therapy is
valuable in patients with 17q deletion and may be
Monoclonal a ntibodies given in combination with alemtuzumab where it
Rituximab (anti - CD20) is valuable in combination can be effective in reducing lymphadenopathy or

with chemotherapy and for treatment of autoim- splenomegaly. Corticosteroids are also indicated in
mune cytopenias. Alemtuzumab (anti - CD52) is a autoimmune haemolytic anaemia, thrombocytope-
monoclonal antibody that is highly eff ective at nia and red cell aplasia.
killing B and T lymphocytes by complement fi xa- Lenalidomide is a thalidomide derivative (see p.
tion. It is given intravenously or subcutaneously 175 ) and has therapeutic activity in CLL. Initial
over several weeks either alone or with corticoster- treatment is sometimes associated with a disease
oids and is valuable in resistant and relapsed disease. ‘ flare ’ at affected tissue sites and the mechanism of

It is also being tested for a potential role in killing action is uncertain.
residual leukaemia cells after R - FC treatment has Bendamustine related to alkylating and anti-
been completed. It is highly immunosuppressive purine drugs is also in trials.
and antibacterial and antiviral prophylaxis is needed.
Another anti - CD20 antibody of atumumab and an Other f orms of t reatment
anti - CD23, lumiliximab are in trial. • Radiotherapy This is valuable in reducing the size

of bulky lymph node groups that are unrespon-
Corticosteroids sive to chemotherapy. Radiotherapy to the spleen
Patients in bone marrow failure may be treated may be valuable in late - stage disease.

initially with prednisolone alone until there is • Combination chemotherapy Cyclophosphamide,

significant recovery of the platelet, neutrophil and hydroxodaunorubicin, oncovin (vincristine) and