Additionally, they found that patients with no previous treatment for the underlying CLL at time of RS transformation had significantly better overall survival compared with patients previously treated for their disease (35.4 months vs 4.2 months, respectively; P <.01).

Twelve patients underwent stem cell transplantation; the median overall survival for these patients was 59.5 months.

“Patients with high-risk features, which are the great majority of patients with DLBCL-type RS, such as clonally related RS, or with previous treatment for CLL, or TP53 disruption, should be considered for transplant,” Dr Abrisqueta said. “However, only a minority of patients are eventually able to receive transplant because a significant percentage of the patients are older or no adequate response is obtained with previous treatments.”


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Dr Hou agreed, adding that transplant consolidation is primarily for people who are younger or who have a very good performance status.

“For patients with CLL, the median age at diagnosis is 70 [years], so at least half of people won’t probably qualify for transplant.” he said.

On the whole, these data suggest that a small subgroup of patients with DLBCL-type RS, including those patients with no clonal relationship between RS and CLL, or those without prior treatment for CLL and no TP53 alterations, presented a better outcome and may obtain prolonged survival with standard chemoimmunotherapy combinations such as R-CHOP, more similar to de novo DLBCL, Dr Abrisqueta said.

That means it is important for clinicians to assess cytogenetics, and look at clonality, according to Dr Hou.

“Knowing the biology of the transformation is very important; patients with clonal evolution or high-risk features as shown in this study have poor outcome[s] [with] CHOP-R,” Dr Hou said. “Early referral for transplant evaluation is appropriate for those patients.”

Reference

Abrisqueta P, Delgado J, Alcoceba M, et al. Clinical outcome and prognostic factors of patients with Richter syndrome: real-world study of the Spanish Chronic Lymphocytic Leukemia Study Group (GELLC) [published online June 9, 2020]. Br J Haematol. doi: 10.111/bjh.16748