An Unusual Presentation of a Chronic Lymphocytic Leukemia Patient with 17p Deletion After Reduced-Intensity Transplantation: Richter Syndrome and Concomitant Graft-Versus-Host Disease—Case Report

• Published in: Transplantation proceedings Vol. 45; no. 7; pp. 2845 - 2848
• Main Authors: Salihoglu, A, Ozbalak, M, Keskin, D, Tecimer, T, Soysal, T, Ferhanoglu, B
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-09-2013
• Subjects: Chromosome Deletion; Chromosomes, Human, Pair 17; Female; Graft vs Host Disease; Humans; Leukemia, Lymphocytic, Chronic, B-Cell - genetics; Leukemia, Lymphocytic, Chronic, B-Cell - therapy; Middle Aged; Stem Cell Transplantation; Surgery; Syndrome
• ISSN: 0041-1345; 1873-2623
• DOI: 10.1016/j.transproceed.2012.12.001

Abstract Chronic lymphocytic leukemia (CLL) patients with 17p deletion comprise a challenging subgroup associated with poor overall survival. These patients should be treated with alternative strategies. Reduced-intensity conditioning (RIC) allogeneic stem cell transplantation (allo-SCT) can achieve long-term remission in this ultra-high-risk CLL group. Herein, we described a CLL patient with 17p deletion who developed Richter syndrome with extranodal involvement of the liver soon after RIC allo-SCT despite apparent acute graft-versus-host disease. The majority of chronic lymphocytic leukemia (CLL) patients respond well to chemoimmunotherapy. Patients who show ultra-high-risk genetics, such as 17p deletions, comprise a challenging subgroup of patients with poor response to chemoimmunotherapy and median life expectancy <2–3 years at the time of first-line treatment. Current treatment approaches for patients with 17p deletion include agents acting independently from the DNA damage pathway, such as alemtuzumab and high-dose corticosteroids. RIC allo-SCT for consolidation can achieve long-term remission in this ultra-high-risk CLL group.1,2 Richter syndrome (RS) represents the clinicopathologic transformation of CLL to an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). RS appearing after allo-SCT can be managed by tapering of immunosuppression, followed by dose-escalated donor lymphocyte infusion titrated to the degree of leukemia response and graft-versus-host disease (GVHD) encountered. Herein, we describe a CLL patient with 17p deletion who developed RS with extranodal involvement of the liver soon after RIC allo-SCT despite apparent acute GVHD (aGVHD).