A Rare Entity of Accelerated Chronic Lymphocytic Leukemia: A Report of Two Cases and Review of Literature  

作　　者：Zahra Kmira Ben Yahya Noura Chembah Wafa Ben Sayed Nesrine Chiba Dorra Bouteraa Walid Zaier Monia Ben Youssef Yosra Haifa Regaieg Khelif Abderrahim Zahra Kmira;Ben Yahya Noura;Chembah Wafa;Ben Sayed Nesrine;Chiba Dorra;Bouteraa Walid;Zaier Monia;Ben Youssef Yosra;Haifa Regaieg;Khelif Abderrahim(Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia;Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia)

机构地区：[1]Department of Clinical Hematology, Farhat Hached University Hospital, Sousse, Tunisia [2]Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia

出　　处：《Health》2023年第8期861-870,共10页健康（英文）

摘　　要：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.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.

关 键 词：Accelerated Chronic Lymphocytic Leukemia Richter Transformation PROGNOSIS Treatment 

分 类 号：R73[医药卫生—肿瘤]