Oct. 29, 2021
The world of chronic lymphocytic leukemia (CLL) — the most common leukemia in the U.S. and the Western world — is changing rapidly as investigators increasingly comprehend the biology and therapy of the disease.
"Our approach to CLL has revolutionized in the last decade due to research," says Wei Ding, M.B.B.S., Ph.D., a hematologist at Mayo Clinic's campus in Rochester, Minnesota. "We understand CLL far better than before."
Most patients with CLL remain symptom-free at diagnosis, thus they do not require therapy per the standard approach. This situation can leave patients speculating about their diagnoses and also trying to determine how to relieve disease-related stress. "Patients start to ask the number of years they can live, and how to plan for future days," says Dr. Ding.
A comprehensive, innovative approach for newly diagnosed patients
Mayo Clinic's CLL specialists saw the need for information and direction in patients recently diagnosed with CLL and sought to fill the gap. The team developed a singular approach for newly diagnosed CLL. The approach, designed to help patients comprehend their prognoses, involves conducting extensive testing followed by counseling and education to help patients grasp the trajectory of their disease.
In local communities, patients often learn they have CLL through bloodwork done with their primary care providers. Through the blood testing, their physicians determine that the white blood cell counts are outside the standard range — typically too high — leading to a CLL diagnosis. At this juncture, patients often are referred to tertiary care centers such as Mayo Clinic.
CCL testing available
With no immediate treatment and a host of unknowns about the future, patients with CLL need a place to turn for help. Mayo Clinic offers patients tools to understand their prognoses, which can alleviate anxiety. "Patients are relieved after testing, and it puts their minds at ease or at least in a normal place," says Dr. Ding. At Mayo Clinic, CLL testing includes:
CLL fluorescence in situ hybridization (FISH) panel
This assay, developed by Mayo Clinic Laboratories, identifies specific abnormalities in CLL cells, indicating risk level.
Immunoglobin heavy locus (IGH) somatic hypermutation analysis, B-cell chronic lymphocytic leukemia (B CLL)
Also developed by Mayo Clinic Laboratories, this assay points to somatic hypermutation status, if present.
Hematologic neoplasms (TP53) somatic mutation, DNA sequencing exons 4-9
A mutation in TP53, a tumor suppressor, occurs in many cancers. In CLL, it has distinct prognostic implications.
CLL monitoring minimal residual disease (MRD) detection
While not yet approved by the Food and Drug Administration, hematologists look to this flow cytometry technique after therapy. It detects the presence of miniscule amounts of disease, with implications for relapse and predicted duration of clinical remission.
Beta-2-microglobulin, serum
Hematologists use this tumor marker result to provide information to patients regarding their risk of disease progression.
CD279 (PD-1) immunostain
Pathologists Rong He, M.D., and Karen L. Rech, M.D., and Dr. Ding, in conjunction with Mayo Clinic Laboratories, developed this easy-to-conduct assay for Richter transformation from CLL. Richter transformation is a process of transforming CLL into an aggressive lymphoma, most commonly diffuse large B cell lymphoma, which affects 5% to 15% of patients with CLL. The presence of PD-1 positive lymphoma cells suggests a related clonal relationship of Richter cells with CLL.
After this battery of tests, the Mayo Clinic CLL team provides counseling and education for patients, allowing them to appreciate their disease in its context. The physicians use the CLL International Prognostic Index to present patients with a 0 to 10 score:
- 0 to 2 — Minimal risk
- 2 to 3 — Low risk
- 4 to 6 — Medium risk
- 7 to 10 — High risk
They also counsel patients regarding immunodeficient status, including the increased risk of infection and skin cancer, and the need for standard vaccinations and cancer prevention. Currently, Dr. Ding strongly encourages patients with CLL to be vigilant with COVID-19 preventive measures. The test panel also provides the CLL specialists with information needed to create an appropriate return visit schedule.
Treatment expertise and resources
If a patient with CLL needs treatment, Mayo Clinic offers many options:
- Mayo Clinic has numerous clinical trials available, and Mayo hematologists have led many CLL trials.
- A tumor board with multiple experts in CLL meets to discuss cases, which Dr. Ding indicates "means we have more power to offer the patient."
- As CLL treatment has evolved from chemotherapy to novel oral therapy, Mayo Clinic Speciality Pharmacy has offered patients numerous services. The pharmacy helps patients look for medication payment assistance, checks for any potential drug interactions and ensures patients don't run out of medications.
Considerations for referring physicians
Dr. Ding highly recommends sending patients with newly diagnosed CLL to a specialty center such as Mayo Clinic. She explains a solely CLL-focused, highly subspecialized group such as Mayo Clinic's is in a unique position to offer comprehensive testing, counseling and education services and provide follow up customized for each patient.
She encourages physicians whose patients' white blood cell counts are not in the normal range to refer, even if a CLL diagnosis has not yet been determined.
When physicians refer patients with diagnosed or suspected CLL, Mayo Clinic specialists maintain close contact with the patient's hometown physician throughout the consult processes and any treatment.
For more information
Test catalog. Mayo Clinic Laboratories.
Clinical trials: Chronic Lymphocytic Leukemia. Mayo Clinic.