Chronic lymphocytic leukaemia
Dr Gem E. George-Tharakan educates us on chronic lymphocytic leukaemia (CLL) and why targeted therapy is the optimal treatment.
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What is chronic lymphocytic leukaemia (CLL)?
CLL is a type of cancer of the blood and bone marrow. Specifically, it’s cancerous growth of B lymphocytes (sub-type of white (fighter) cells produced in the bone marrow that circulate in peripheral blood, mature and function in secondary lymphoid tissue, such as lymph nodes, liver, spleen, etc.).
CLL is a common leukaemia that is typically seen in older patients, however, younger people can also be affected. There may also be a hereditary component where people in the same family have been seen to have an increased incidence, or in conjunction with family members with other low-grade lymphomas.
Many patients may be diagnosed incidentally during annual medical check-ups and on routine blood tests. However, symptomatic patients may present with: swollen, non-painful glands; enlarged spleen; low blood haemoglobin; low numbers of platelets; infections, fatigue; and reduced exercise tolerance. Patients with advanced disease may present with: weight loss; recurrent infections; bleeding; symptomatic anaemia; not commonly night sweats, fevers (B-symptoms), etc.
There are also less common presentations, with extra nodal (not in typical lymphoid tissue) involvement, such as an infiltration of the scalp, prostate, gonads, pharynx, lungs and gastrointestinal tract.
A full blood count shows an increase in the total white cell count and/or a raised absolute lymphocyte count of greater than 5.0 X 109/L. The blood smear made on a slide when reviewed shows an increase in normal-looking lymphocytes. In some patients, a reduction in the red cells and platelets may also be seen due to immunological complications of the condition, or due to disease burden.
Certain biochemical tests may also show abnormalities; however, this isn’t always the case. During the follow-up of this condition, these tests are also routinely performed. Screening for viral infections also needs to be done to prevent reactivation during therapy.
The gold-standard for the diagnosis is a special molecular test known as flow cytometry. This test helps distinguish normal B lymphocytes from malignant B lymphocytes through the preferential expression of abnormal markers on the B lymphocytes. Part of the work-up may include a bone marrow biopsy and further molecular and genetic testing. This helps determine the disease burden and assists in the prognosis of the condition and helps to determine possible treatment options.
The clinical staging is completed by conducting radiological imaging, such as CT scans. It’s based on the Rai and Binet staging systems. Biochemical test results and genetics help complete the classification of the patient into low-, intermediate-, high- or very high-risk categories.
Treatment supportive and non-chemotherapeutic
Watch and wait is a common treatment approach in patients who are asymptomatic. Supportive treatment may include transfusion of blood products, antibiotics, etc.
This is determined by various factors including patient factors (co-morbidities, chronic medication, end-organ damage, performance status, patient preference, treatment goals), disease factors (disease burden and/or its complications) and genetic factors (del17p, TP53 mutations, IGHV mutational status), etc.
Combinations of treatments
Due to the advancements in the field of CLL, there are now several therapeutic options available which may include combinations of traditional chemotherapeutic agents, in conjunction with immunotherapy, such as monoclonal antibodies to CD20, and targeted therapy, such as B-cell lymphoma 2 (Bcl2) and bruton tyrosine kinases (BTKi) inhibitors.
Targeted therapy is the optimal treatment
Targeted therapy is the optimal treatment when compared to traditional chemotherapy because it specifically targets the cancer cells and not normal, healthy cells. This limits side effects and improves tolerance to treatment.
The benefit of the above advancements is that clinicians in private practice and medical aids in SA can offer patients various treatment options, such as a fixed duration combinations versus continuous therapy, which would allow someone with CLL to get treatment as an outpatient, in the comfort of their homes. This limits clinic visits and avoids chair-time with more manageable side effect profiles.
MEET THE EXPERT – Dr. G.E.George-Tharakan
Dr. G.E.George-Tharakan (MBChB (WSU/UNITRA), FCPath Haem(SA), MMed Haem(UP), Certificate in Clinical Haematology (SA)) is a clinical haematologist working with Drs Brittain and Partners, ABJ Oncology. Having lived and studied in four of the nine provinces in SA, during her career thus far, she understands the frustrations in accessibility and availability of quality care for haematology patients and hopes to do her part in bridging that gap.
This article is sponsored by AstraZeneca in the interest of education, awareness and support. The content and opinions expressed are entirely of the patient and not influenced by AstraZeneca in any way.
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