Chronic lymphocytic leukemia (CLL) is a hematological malignancy characterized by the proliferation of mostly mature but abnormal leukocytes.
CLL is considered the most common type of leukemia in the Western hemisphere; its prevalence in Europe and North America ranges from 29-38% of all leukaemias 1. It primarily affects adults ~65-70 years of age 3.
Up to half of the patients can be asymptomatic with the disease being incidentally discovered by routine blood work up 3. Patients may sometimes present with hepatomegaly, splenomegaly or both and/or hemolytic anemia.
It is a B-lineage neoplasm of pre-follicular center cells that is usually associated with circulating neoplastic small lymphocytes. From a morphologic and immunophenotypic perspective, the malignant cells of CLL tend to be identical to those of nodal-based small lymphocytic lymphoma (SLL), and these two malignancies are thought to represent different manifestations of the same disease 1.
There is two common staging system in use which are:
The diagnosis is generally established by a bone marrow biopsy and immunophenotyping.
As with other types of bone marrow infiltrative disease, it is possible to see a diffusely hypointense signal of bone structures on T1, easier to see on spinal MRI.
Treatment and prognosis
CLL treatment is offered in the presence of active disease, which is characterized by any of the listed below 7:
- presence of B symptoms
- progressive bone marrow failure
- extensive or rapidly progressing lymphadenopathy
- massive or rapidly progressive splenomegaly
- uncontrolled autoimmune cytopenias
- rapid lymphocyte doubling time: either occurring in less than 6 months or when there is an increase in 50% in ≤2 months
About one-third of patients with CLL will never require treatment 7.
Richter transformation: is defined as a diffuse large cell lymphoma, occurring by transformation of chronic lymphocytic leukemia 2,4.
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- 2. Ghofrani M, Tantiwongkosi B, Smith AS et-al. Richter transformation of chronic lymphocytic leukemia presenting as a dural-based non-hodgkin lymphoma mass. AJNR Am J Neuroradiol. 2007;28 (2): 318-20. AJNR Am J Neuroradiol (full text) - Pubmed citation
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- 4. Tardif S, De kerviler E, Chaibi P et-al. CT and MR patterns of spinal involvement in Richter syndrome. J Comput Assist Tomogr. 19 (1): 146-9. - Pubmed citation
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- 7. Girish S. Shroff, Mylene T. Truong, Brett W. Carter, Marcelo F. Benveniste, Rashmi Kanagal-Shamanna, Greg Rauch, Chitra Viswanathan, Prajwal C. Boddu, Naval Daver, Carol C. Wu. Leukemic Involvement in the Thorax. (2019) RadioGraphics. 39 (1): 44-61. doi:10.1148/rg.2019180069 - Pubmed