Canterbury DHB
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The WHO criteria require that prolymphocytes comprise more than 55% of circulating lymphoid cells. Prolymphocytic leukaemia (PLL) may arise de novo, or may develop in the end stages of CLL.
PLL usually presents with marked splenomegaly and large numbers of prolymphocytes in the blood.
Unlike CLL cells, they express strong surface immunoglobulin and are more often FMC7 positive and are CD23 negative. Only about one third of cases are CD5 positive. Some PLL are of T-cell origin and this is more likely to be found in Asians.
Responses to fludarabine, cladribine and alemtuzimab (anti-CD52, Campath®), may be seen, but the outlook is poor with median survival in the order of 12-18 months.
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Topic Code: 5521
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