Drs. Ravi Vij and Alvaro Alencar explain the staging process of CLL and its impact on treatment decisions. Watch their interview below: 

The Rai staging system is commonly used in the United States to understand the severity of a patient's CLL. The Rai system might seem like how other cancers are graded, but CLL is different. Dr. Alencar stated that many patients ask if the disease has spread (metastasized). But with CLL, the cancer cells are already throughout the blood from the start. This is different from other cancers where the grading shows where the cancer is located. In CLL, the Rai staging system shows how serious the disease is. 

Rai Staging System in CLL

The Rai staging system categorizes CLL into five stages:

• Rai stage 0: Many lymphocytes (white blood cells) with no other issues
• Rai stage I: Lymph glands are swollen
• Rai stage II: Spleen and/or liver are enlarged
• Rai stage III: Low numbers of red blood cells (anemia)
• Rai stage IV: Low numbers of platelets (thrombocytopenia)

Why Staging Matters

Staging helps doctors predict disease progression and decide on potential treatments. Treatment is typically considered if a patient progresses to Rai stage II or beyond. Other reasons to consider treatment include rapid white blood cell count increases, or the manifestation of symptoms such as fevers, night sweats, and weight loss.

Other factors that predict CLL progression 

Apart from Rai stages, the genetic features of CLL cells can influence how the disease progresses. These factors may be added to future CLL staging systems. Two of the main genetic mutations doctors look for in CLL cells are: 

• Immunoglobulin Heavy Gene Mutation (IGHV): If the IGHV gene is mutated, the CLL cell is older and the disease typically progresses slowly. If IGHV is unmutated, the CLL cell is young and the disease may progress quickly 
• Del(17p)/TP53 mutation: The TP53 gene (located on chromosome 17) is in charge of creating protein 53 (p53) which helps suppress cell tumor growth, repair cell DNA, and instructs cells beyond repair to die. If CLL patients have either a deletion or mutation of the TP53 gene, they may require treatment at the time of diagnosis as the CLL cells can multiply more quickly than CLL cells without the TP53 deletion/mutation 

CLL patients can talk with their CLL specialist about any concerns they have related to the stage of their disease and their treatment options.