A 45-year-old gentleman with no known medical illness presented to the hospital with a two-week history of fever, symptomatic anaemia, abdominal distension, and multiple lymphadenopathies. Complete blood count showed leukocytosis of 43.7 × 109/L, haemoglobin 2.5 g/dL, and platelet 59 × 109/L. Lactate dehydrogenase (LDH) was 747 U/L (range < 250 U/L), and Coombs’ test was negative . The haptoglobin test was not available in the hospital. Peripheral blood film revealed 96% small mature lymphocytes, 4% prolymphocytes, and numerous smudge cells. Bone marrow smears reported hypercellular marrow with lymphocytosis. The lymphocytes were small to medium in size with clumped chromatin and inconspicuous nucleoli. The immunophenotyping (IP) showed 58% of lymphocytes expressed CD19+, CD5+, CD23+, CD25+, FMC7–, and lambda restriction. There were 17p13.1 and 13q14.2 deletions from FISH analysis. Contrast-enhanced computed tomography (CECT) of the neck, thorax, abdomen, and pelvis showed extensive lymphadenopathies in bilateral cervical, supraclavicular, paratracheal, paraaortic, and inguinal with mild splenomegaly (13.6 cm). The biggest lymph node was found at the right cervical, measured 35 × 16 × 69 mm. The diagnosis of CLL Rai IV, Binet C with del(17p) and del(13q) was established.
Author(s) Details:
Ching Soon Teoh
Haematology Unit, Department of Internal Medicine, Hospital Pulau Pinang, Jalan Residensi, 10990
Georgetown, Penang, Malaysia.
Ai Sim Goh
Haematology Unit, Department of Internal Medicine, Hospital Pulau Pinang, Jalan Residensi, 10990
Georgetown, Penang, Malaysia.
Recent Global Research Developments in Chronic Lymphocytic Leukaemia: Challenges in Treatment
Current Treatment Strategies:
- Treatment decisions for CLL patients depend on symptoms and risk categories (high, medium, or low risk).
- Genetic markers, including del(17p), del(11q), TP53 dysfunction, and IGHV mutations, impact prognosis.
- Emerging therapies target Bruton tyrosine kinase (BTK), B-cell leukemia/lymphoma 2 (BCL-2), and PI3Kδ.
- Novel oral targeted therapies, often combined with anti-CD20 antibodies, have replaced chemoimmunotherapy [1].
Educational Gaps and Challenges:
- Oncologists and hematologists face challenges in treating relapsed/refractory CLL and mantle cell lymphoma (MCL) in different countries [2] .
- Disparities in treatment approaches and patient outcomes exist due to scattered information[3] .
Clinical Progress and Questions:
- CLL management has evolved significantly with novel therapies, including BTK, BCL-2, and PI3Kδ inhibitors.
- Real-world data reveal treatment patterns and outcomes in patients receiving first-line CLL treatment [4,5].
References
- Patel, K., Pagel, J.M. Current and future treatment strategies in chronic lymphocytic leukemia. J Hematol Oncol 14, 69 (2021). https://doi.org/10.1186/s13045-021-01054-w
- Shah, N. N., Peloquin, S., Cymbalista, F., Dreyling, M., Murray, S., Fiacco, R. D., & Lazure, P. (2022). Challenges in Treatment Decisions and Patient-Provider Communication in Relapsed/Refractory Chronic Lymphocytic Leukemia and Mantle Cell Lymphoma. Blood, 140(Supplement 1), 13106-13108.
- Alhuraiji, A., Alhejazi, A., Samra, M., Bagal, B., Pavlovsky, M. A., Roa, M., … & Gonzalez Hernandez, F. (2023). A Multicenter Retrospective Study to Understand the Clinical Characteristics, Treatment Patterns, and Resource Utilization for Patients with Chronic Lymphocytic Leukemia: CREEK Study. Blood, 142(Supplement 1), 3277-3277.
- Hallek, M., Shanafelt, T. D., & Eichhorst, B. (2018). Chronic lymphocytic leukaemia. The Lancet, 391(10129), 1524-1537.
- Mato, A. R., Ravelo, A., To, T. M., Schuldt, R., & Biondo, J. M. (2021). Real-world treatment patterns and outcomes of patients with chronic lymphocytic leukemia (CLL) receiving first-line (1L) therapy in the United States (US). Blood, 138, 4086.