Traditional estimates of Richter's transformation range from 1% to 8%. The CLL clone can transform into an acute lymphoma, similar to a diffuse B cell lymphoma. Hodgkin's disease can also arise in CLL patients. Transformation occurs almost exclusively in the unmutated patient.
The DBCL or Hodgkin's can also arise 'de novo', which means it just pops up, and it's unrelated to the CLL cell. In other words, it doesn't transform from the CLL cells, but is a new cancer (secondary malignancy) altogether.
The latest I've read suggest that the incidence can be viewed as an increasing probability as the disease progresses over time. The rate estimated is about one percent per year in unmutated patients. That means if one lives for 10 years with CLL, there is a 10 % change the clone will transform into Richter's.
All of the amazing news coming in CLL is wonderful, but there is little progress in treating Richter's. I'm going to concentrate my charitable giving on Richter's, as the options are poor, and I'm at risk.
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