NCCN Guidelines for Patients® | Chronic Lymphocytic Leukemia
45 NCCN Guidelines for Patients ® : Chronic Lymphocytic Leukemia, 2018 4 Treatment guide Richter’s transformation and CLL with progression If you can’t join a clinical trial, ibrutinib is the standard of care. Venetoclax with or without rituximab is another approved option for patients with CLL with del(17p) and who have received prior treatment. Idelalisib can also be given alone or with rituximab as treatment. Other options include HDMP with rituximab, lenalidomide with or without rituximab, alemtuzumab with or without rituximab, and ofatumumab. If you have a complete or partial response to treatment for refractory or relapsed disease (after second-line therapy), your doctor may consider a second maintenance therapy with lenalidomide or ofatumumab. Your doctor will test for MRD and decide on your next steps based on those results. It is helpful to ask your doctor to explain any further treatment options you may have. Richter’s transformation and CLL with progression About 2 to 10 out of 100 people with CLL will have a change occur in the cells, a transformation that causes a new type of cancer. This is called Richter’s transformation. Richter’s transformation is when CLL becomes a more aggressive lymphoma such as DLBCL or Hodgkin lymphoma. A lymphoma is a hematologic cancer–like leukemia–and this cancer starts in our lymphatic system. If there is any concern that CLL has transformed, your doctor will recommend additional tests. This may include blood tests as well as imaging such as a PET scan. The PET scan can help your doctor see if it looks like there may be a Richter’s transformation. It can also find the best location to perform a biopsy. The type of biopsy you will have depends on where the lymph node is located and if it can be easily reached. Types of biopsies include an FNA ( f ine- n eedle a spiration [small sample is taken]), core needle biopsy (large sample of tissue is taken), and excisional biopsy (removes the whole lymph node). An excisional biopsy is advised if possible. CLL can also progress and become a more aggressive disease. Progression is the growth or spread of cancer. Thus, your doctor may look at the cells under a microscope to assess for changes (expanded proliferation centers) and study how quickly the cells are growing. A blood test may be done to check for increased prolymphocytes (type of white blood cell) in the blood. This progression is not Richter’s transformation. Your doctor will consider your next steps of care if the disease progresses.