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Chapter 18 Chronic lymphoid leukaemias / 241

prednisolone (CHOP) with rituximab is some- disease and recurrent infection. The disease may
times used in late - stage cases. transform into a localized high - grade lymphoma

’
• Bendamustine is both an alkylating agent and (Richter s transformation) or there may be the
purine analogue. In trials it is combined with appearance of an increasing number of prolym-
rituximab. phocytes that are resistant to treatment.

• Ciclosporin Red cell aplasia may respond to
ciclosporin.

B - c ell p rolymphocytic l eukaemia
• Splenectomy This is generally reserved for those

patients with immune - mediated cytopenias that Although prolymphocytic leukaemia (PLL) may
do not respond to short courses of steroids or initially appear similar to CLL, the diagnosis is
those with painful bulky enlargement of the made by the appearance of a majority of prolym-

spleen not responding to other therapy. phocytes in the blood. The prolymphocyte is around
• Immunoglobulin replacement Immunoglobulin twice the size of a CLL lymphocyte and has a large
(e.g. 400 mg/kg/month by intravenous infusion) central nucleolus (Fig. 18.6 ). B - cell PLL is three

is useful for patients with hypogammaglobulinae- times more common than T - cell PLL.
mia and recurrent infections, especially during PLL and CLL also differ in their clinical features.

winter months. PLL typically presents with splenomegaly without
• Stem cell transplantation This is currently an lymphadenopathy and with a high and rapidly rising

experimental approach in younger patients. lymphocyte count. Anaemia is a poor prognostic

Allogeneic stem cell transplantation (SCT) may feature. Treatment is difficult in PLL. Splenectomy

be curative but has a high mortality rate. is usually of benefit and purine nucleoside analogues,
rituximab or alemtuzumab may help.
Course of d isease
Hairy c ell l eukaemia
Many patients in Binet stage A or Rai stage 0 or I
never need therapy and this is particularly likely for Hairy cell leukaemia (HCL) is an uncommon
those with favourable prognostic markers (Table B-cell lymphoproliferative disease with a

18.3 ). For those who do need treatment a typical male : female ratio of 4 : 1 and a peak incidence at

pattern is that of a disease that is responsive to 40 – 60 years. Patients typically present with infec-
several courses of chemotherapy before the gradual tions, anaemia or splenomegaly. Lymphadenopathy
onset of extensive bone marrow infi ltration, bulky is very uncommon. Pancytopenia is usual at pres-

Figure 18.6 Prolymphocytic
leukaemia: blood fi lm showing
prolymphocytes that have
prominent central nucleoli and an
abundance of pale cytoplasm.