Richter syndrome (RS) is an uncommon complication of chronic lymphocytic leukemia (CLL) associated with a dismal prognosis and a median survival of 1 year or less.

Given how rare RS is, prognostic factors and strategies for best management of these patients remains to be elucidated, according to Pau Abrisqueta, MD, of Hospital Vall d’Hebron, Spain.

To gain more insight into RS, Dr Abrisqueta and colleagues from the Spanish Chronic Lymphocytic Leukemia Study Group recently published their analysis of real-world data detailing the clinical outcomes and prognostic factors of 128 patients diagnosed with the syndrome between 1988 and 2017.

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Patients included 112 with diffuse large B-cell lymphoma (DLBCL)-type RS, 15 with Hodgkin lymphoma (HL)-type RS, and 1 case with plasmablastic lymphoma. There was a large difference in median overall survival between those with DLBCL-type RS (5.9 months) and those with HL-type RS (20.8 months).

“Previously reported data already pointed at the same direction as what we observed in our series, with better outcome of HL-type RS than that observed in the DLBCL-type RS, consistent with the notion that most HL-type RS are probably secondary tumors unrelated to the CLL than true transformations, which is the case of most DLBCL-type RS cases,” Dr Pau said.

When considering RS, one of the key questions is of clonality, explained Jing-Zhou Hou, MD, of UPMC Hillman Cancer Center in Pittsburgh, Pennsylvania. In DLBCL-type RS, there are 2 categories: the first is clonal evolution from the CLL clones to a more aggressive lymphoma, and the second is de novo RS, which has different clonality from CLL.

“De novo RS have better outcomes,” Dr Hou said. “Eighty-percent of RS has features of clonal evolution and these have poor outcomes.”

Indeed, Dr Abrisqueta and colleagues found that — among other factors — TP53 abnormalities in the CLL clone, and clonal relationship between CLL and RS, were associated with overall survival in patients with DLBCL-type RS.