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.
Transplant Proc
; 45(7): 2845-8, 2013 Sep.
Article
en En
| MEDLINE
| ID: mdl-23747187
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).
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Cromosomas Humanos Par 17
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Leucemia Linfocítica Crónica de Células B
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Deleción Cromosómica
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Trasplante de Células Madre
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Enfermedad Injerto contra Huésped
Límite:
Female
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Humans
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Middle aged
Idioma:
En
Revista:
Transplant Proc
Año:
2013
Tipo del documento:
Article
País de afiliación:
Turquía