Management

• PLL is typically resistant to chlorambucil.

• Splenectomy: may be symptomatically helpful, 'debulking', follow up with other therapy.

• Splenicirradiation: offers symptomatic relief if unfit for splenectomy.

• Combination chemotherapy: CHOP may achieve responses in about 33% and prolong survival in younger patients.

• Purine analogue therapy: Fludarabine, 2-CDA or deoxycoformycin may produce responses in some patients.

• Campath-1H: anti-CD52 monoclonal antibody produces responses in both B-PLL and T-PLL. In T-PLL CR rates of 40-60% have been achieved lasting several months and permitting subsequent high dose therapy with autologous or allogeneic SCT and prolonged survival.

• Rituximab: anti-CD20 monoclonal antibody: there are reports of responses in B-PLL.

• Stem cell transplantation: in view of the poor prognosis of PLL, younger patients who achieve a CR should be considered for allogeneic SCT where possible or autologous SCT.

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