Related Experiment Videos
Chronic lymphocytic leukemia in (Richter's) transformation
F J Giles1, S M O'Brien, M J Keating
1Division of Medicine, MD Anderson Cancer Center, Houston, TX 77030, USA.
Seminars in Oncology
|March 3, 1998
Summary
Richter's syndrome (RS), an aggressive non-Hodgkin's lymphoma (NHL), can develop in chronic lymphocytic leukemia (CLL) patients. Continued surveillance is crucial as RS occurs even with modern CLL treatments.
Area of Science:
- Hematology
- Oncology
- Clinical Medicine
Background:
- Chronic lymphocytic leukemia (CLL) patients have an increased risk of developing secondary malignancies, including non-Hodgkin's lymphoma (NHL), Hodgkin's Disease (HD), and multiple myeloma (MM).
- Richter's syndrome (RS) is defined as the development of aggressive NHL in patients with pre-existing CLL, often presenting with abrupt clinical deterioration and rapid tumor growth.
- The exact incidence of these secondary malignancies may be underestimated due to the lack of routine postmortem examinations.
Purpose of the Study:
- To investigate the incidence, clinical presentation, diagnosis, and clonal evolution of secondary malignancies, particularly Richter's syndrome, in patients with chronic lymphocytic leukemia.
- To assess the relationship between CLL and aggressive NHL in Richter's syndrome, including common or distinct clonal origins.
- To evaluate the impact of current therapies on the occurrence and behavior of secondary malignancies in CLL patients.
Main Methods:
- Review of patient data and clinical outcomes for individuals diagnosed with chronic lymphocytic leukemia.
- Histopathological and immunophenotypic analysis of tissue biopsies for diagnosis of secondary malignancies.
- Gene rearrangement studies and isotype analysis to determine clonal origins of co-existing CLL and NHL.
Main Results:
- Non-Hodgkin's lymphoma (NHL) develops in approximately 3% of CLL patients, with Richter's syndrome being the aggressive form.
- Diagnosis of RS requires tissue biopsy, typically revealing diffuse large cell lymphoma (LCL) or its immunoblastic variant, which is often resistant to therapy with a median survival of 6 months.
- Studies suggest a common clonal origin for CLL and LCL in RS patients in about 60% of cases, with distinct origins in 40%.
Conclusions:
- Richter's syndrome remains a significant concern in CLL patients, necessitating ongoing surveillance despite advances in CLL treatment.
- Secondary malignancies like Hodgkin's Disease and multiple myeloma in CLL patients are often diagnosed at advanced stages with poor prognoses.
- Purine analog therapy for CLL does not appear to influence the incidence or clinical course of Richter's syndrome.