Abstract
CLL is usually an indolent lymphoproliferative disease; however pts are at risk over time (3-5% yr) of transformation to a more aggressive histology, e.g., prolymphocytic leukemia (PLL), or Richter’s transformation (RT). The outcome of these pts is poor with median overall responses of 5–43%, CR 5–43%, and overall survival of 5–8 months regardless of the chemotherapeutic approach.
We report on 15 pts with CLL transformed to PLL or RT treated at our institution over the last 4 years. The median pt age at transformation was 64 (51–79). 9 had PLL, 4 had RT, one had an anaplastic histology, and another with atypical transformation. 7/14 pts were positive for ZAP-70 expression. 10/14 presented with CLL prior to the transformation with a median time of 7 yrs (range 3–27), and had undergone prior therapy with either fludarabine or chlorambucil. Pts were treated with mainly rituximab chemotherapy combinations. One pt who achieved a CR, was consolidated with a matched related donor transplantation and remains disease free for 3 yrs.
Conclusion. CR and ORR were higher than reported in the literature. Also, pts treated with rituximab-based combinations achieved longer overall survivals. Rituximab based-combinations should be considered in treating such pts. Whether they would benefit from consolidation therapy needs further investigation.
Treatment . | # Pts Prev. Tx . | # Pts . | CR (%) . | PR (%) . | OS after Transformation (Months) . |
---|---|---|---|---|---|
Pts, patients; Prev., previously; Tx, treated; CR, complete response; PR, partial response; OS, overall survival; R, rituximab; Flu, fludarabine; Cy, cyclophosphamide | |||||
PLL | |||||
R/Flu +/− Cy | 2/6 | 6 | 67 | 12 | 36+, 36+, 36, 24+, 4, 12+ |
RCHOP or R-EPOCH | 2/2 | 2 | 0 | 50 | 8, 3+ |
Alemtuzumab | 1/1 | 1 | 0 | 100 | 12+ |
RT | |||||
RCHOP or REPOCH | 1/4 | 4 | 75 | 25 | 36+, 30+, 3, 9 |
Anaplastic | |||||
RCHOP | 1/1 | 1 | 100 | 0 | 24+ |
Atypical | |||||
R/Flu/Cy | 1/1 | 1 | 100 | 0 | 24+ |
Overall | 60 | 27 |
Treatment . | # Pts Prev. Tx . | # Pts . | CR (%) . | PR (%) . | OS after Transformation (Months) . |
---|---|---|---|---|---|
Pts, patients; Prev., previously; Tx, treated; CR, complete response; PR, partial response; OS, overall survival; R, rituximab; Flu, fludarabine; Cy, cyclophosphamide | |||||
PLL | |||||
R/Flu +/− Cy | 2/6 | 6 | 67 | 12 | 36+, 36+, 36, 24+, 4, 12+ |
RCHOP or R-EPOCH | 2/2 | 2 | 0 | 50 | 8, 3+ |
Alemtuzumab | 1/1 | 1 | 0 | 100 | 12+ |
RT | |||||
RCHOP or REPOCH | 1/4 | 4 | 75 | 25 | 36+, 30+, 3, 9 |
Anaplastic | |||||
RCHOP | 1/1 | 1 | 100 | 0 | 24+ |
Atypical | |||||
R/Flu/Cy | 1/1 | 1 | 100 | 0 | 24+ |
Overall | 60 | 27 |
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