title: 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.
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).(3) 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.(4) 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).
authors.affiliation: Division of Hematology, Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors.forename: A
authors.initials: A
authors.lastname: Salihoglu
authors.forename: M
authors.initials: M
authors.lastname: Ozbalak
authors.forename: D
authors.initials: D
authors.lastname: Keskin
authors.forename: T
authors.initials: T
authors.lastname: Tecimer
authors.forename: T
authors.initials: T
authors.lastname: Soysal
authors.forename: B
authors.initials: B
authors.lastname: Ferhanoglu
mesh_terms: D002872:Chromosome Deletion; D002886:Chromosomes, Human, Pair 17; D005260:Female; D006086:Graft vs Host Disease; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000235:genetics / Q000628:therapy*; D008875:Middle Aged; D033581:Stem Cell Transplantation; D013577:Syndrome