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A Rare Entity of Accelerated Chronic Lymphocytic Leukemia: A Report of Two Cases and Review of Literature
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 1 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 2 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 3 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 4 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 5 Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia
- 6 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 7 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 8 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 9 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
- 10 Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia
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Abstract
Background: Accelerated-chronic lymphocytic leukemia (A-CLL) is a rare disease entity as it represents less than 1% of all reported cases of chronic lymphoid leukemia (CLL). Moreover, it is most likely an under diagnosed entity due to its rarity and the non-standardized practice of lymph node biopsy in CLL. Purpose: The aims of our work are to establish the diagnosis of A-CLL and to study the prognosis and treatment of this rare entity. Method: here, we report the clinical presentation and the follow up of two cases of A-CLL. Results: Distinguishing Richter transformation (RT) from A-CLL is important as it may result in a major change in disease management. The prognosis of A-CLL is intermediate between CLL and RT. The prognosis is mainly poor due to a predominance of poor prognostic markers including an increasing number of p53-positive cases. Conclusion: To this date, no prospective study has been led to define the best treatment for A-CLL. The shorter survival of A-CLL when compared to typical CLL implies the need of a more aggressive treatment.
KeywordsAccelerated Chronic Lymphocytic LeukemiaRichter TransformationPrognosisTreatment
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