Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
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Advances in Clinical and Experimental Medicine

2018, vol. 27, nr 12, December, p. 1683–1689

doi: 10.17219/acem/75903

Publication type: original article

Language: English

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Richter syndrome: A rare complication of chronic lymphocytic leukemia or small lymphocytic lymphoma

Ewa Wąsik-Szczepanek1,A,B,C,D,E,F, Agnieszka Szymczyk1,2,A,B,C,D,F, Dariusz Szczepanek3,A,B,C,D,E,F, Joanna Wszoła-Kleinrok4,B,C,D,F, Sylwia Chocholska1,B,C,F, Andrzej Pluta5,B,C,D,E,F, Marek Hus1,A,B,C,D,E,F

1 Chair and Department of Hematooncology and Bone Marrow Transplantation, Medical University of Lublin, Poland

2 Independent Clinical Transplantology Unit, Medical University of Lublin, Poland

3 Chair and Department of Neurosurgery and Pediatric Neurosurgery, Medical University of Lublin, Poland

4 Department of Nephrology, Endocrinology, Hypertension and Internal Diseases of Independent Public Hospital in Zamość, Poland

5 Department of Hematooncology , Oncology Centre, Brzozów, Poland

Abstract

Background. Richter’s syndrome (RS) is a rare complication with an unfavorable prognosis, in which chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) transform into a more aggressive type of lymphoma, most commonly into diffuse large B cell lymphoma (DLBCL) or less often into Hodgkin’s lymphoma (HL).
Objectives. The objective of this research paper was to present a retrospective analysis of patients with CLL/SLL whose disease transformed into RS.
Material and Methods. The study included 217 patients (100 women and 107 men) with CLL/SLL diagnosed in the years 2006–2015 at the Department of Hematooncology and Bone Marrow Transplantation of the Medical University of Lublin, which transformed into RS. We analyzed clinical, laboratory, immunophenotypic (ZAP-70 and CD38 expression), histopathological, and genetic data (del(17p), del(11q)), which was collected at the time of CLL/SLL diagnosis, and some which was collected at the time of transformation.
Results. Richter’s syndrome was diagnosed in 4.6% of all CLL and SLL patients. The group of patients with RS consisted of 9 patients with primary CLL and 1 patient with a diagnosis of SLL (8 patients with transformation into DLBCL and 2 patients with transformation into HL). Leukemic lymphocytes showed evidence of peripheral blood lymphocyte membrane expression of ZAP70+/CD38+ (1 patient), of ZAP-70+/CD38– (3 patients), of ZAP-70–/CD38– (1 patient), and of ZAP-70–/CD38+ (5 patients). The deletion of 11q (del(11q)) was documented in 2 patients. In 4 cases, the location of RS was extremely rare (the thyroid gland, liver, skin, bladder, and central nervous system).
Conclusion. Richter’s syndrome is a rare, but probable complication of CLL/SLL with an unfavorable prognosis, and it should be taken into account at every stage of the disease, particularly when the course of the disease is aggressive.

Key words

chronic lymphocytic leukemia, Richter’s syndrome, small lymphocytic lymphoma

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