Background. Post-transplant lymphoproliferative disorders (PTLD) represent a heterogenous group of disease ranging from reactive polyclonal hyperplasia to aggressive non-Hodgkin lymphomas (NHL) and in the majority of cases they are associated with Epstein-Barr virus (EBV). PTLD develop as a consequence of the severe impairment of T cell function and the related reduction of T cell control on EBV latently infected B cell due to immusoppressive therapy after solid organ (SOT) or hematopoietic stem cell transplantation (HSCT). Aims. We analyzed the clinical features, treatment and outcome of a series of adult patients who developed NHL after liver and kidney transplantation. Methods. We retrospectively studied the clinical data of 16 patients that developed NHL occurred at our institution between 1998 and 2010. Results. 16 patients (M/F ratio 2.2) with a median age of 42 years (range 20-59) developed NHL after liver, 3 cases, and kidney, 13 cases, respectively. Previous liver diseases were: hepatitis C virus cirrhosis in two cases and primary sclerosing cholangitis in one case; previous kidney diseases were: glomerulonephritis in 10 cases and congenital syndrome in three cases. Median time from transplantation to PTLD was 111.5 (range 3-360) months. Six patients experienced acute transplant rejection that required intensification of immunosuppressive therapy. Hematologic diagnosis included 12 monomorphic PTLD (10 diffuse large B lymphoma, 1 peripheral T cell lymphoma, 1 anaplastic large cell lymphoma), 2 classical Hodgkin lymphoma-type PTLD, 1 lymphoplasmacytic lymphoma and 1 extranodal marginal zone lymphoma. Histologic diagnosis was made on lymph node and on extranodal tissue in seven and nine cases, respectively. Available histologic analysis of tumor tissue demonstrated that EBV was positive in 62% of cases. CD20 expression was available in all but two patients and was positive in 71% of cases. At diagnosis seven patients were in stage IV, two in stage III, one in stage II and 6 in stage I; median IPI and Mayo prognostic score were 2 and 1, respectively. Ten patients presented with extranodal disease; central nervous system (CNS) involvement was not observed. Eleven patients received immuno-chemotherapy (including anthracyclines) with rituximab and two only chemotherapy; among them only four patients required reduction of doses because of liver/renal toxicity, and one patient experienced infective toxicity (WHO grade 1-2). One patient was treated with only radiotherapy and two patients were observed because of indolent disease. After a median follow up of 51 months (range 1-128) from the end of therapy, 13 patients are alive and in complete remission, two are lost to follow up and one patient died for progressive disease.CONCLUSIONS: Although the limited number of patients involved, and the peculiar subset of renal and liver allograft in which the risk of PTLD is low-intemediate, our study demonstrated that the majority of our patients developed aggressive NHL and an immuno-chemoterapic regimen is feasible, well tolerated and allow to obtain an elevated percentage of remission.

Post-transplant lymphoproliferative disorders in liver and kidney recipients : a single institution experience / A. Ferrario, D. Vincenti, F. Rossi, N. Orofino, B. Olivero, M. Goldaniga, L. Cro, L. Baldini. - In: HAEMATOLOGICA. - ISSN 0390-6078. - 96:suppl. 2(2011 Jun 09), pp. 563-563. ((Intervento presentato al 16. convegno Congress of the European Hematology Association tenutosi a London nel 2011.

Post-transplant lymphoproliferative disorders in liver and kidney recipients : a single institution experience

A. Ferrario
Primo
;
D. Vincenti
Secondo
;
F. Rossi;N. Orofino;B. Olivero;L. Baldini
Ultimo
2011-06-09

Abstract

Background. Post-transplant lymphoproliferative disorders (PTLD) represent a heterogenous group of disease ranging from reactive polyclonal hyperplasia to aggressive non-Hodgkin lymphomas (NHL) and in the majority of cases they are associated with Epstein-Barr virus (EBV). PTLD develop as a consequence of the severe impairment of T cell function and the related reduction of T cell control on EBV latently infected B cell due to immusoppressive therapy after solid organ (SOT) or hematopoietic stem cell transplantation (HSCT). Aims. We analyzed the clinical features, treatment and outcome of a series of adult patients who developed NHL after liver and kidney transplantation. Methods. We retrospectively studied the clinical data of 16 patients that developed NHL occurred at our institution between 1998 and 2010. Results. 16 patients (M/F ratio 2.2) with a median age of 42 years (range 20-59) developed NHL after liver, 3 cases, and kidney, 13 cases, respectively. Previous liver diseases were: hepatitis C virus cirrhosis in two cases and primary sclerosing cholangitis in one case; previous kidney diseases were: glomerulonephritis in 10 cases and congenital syndrome in three cases. Median time from transplantation to PTLD was 111.5 (range 3-360) months. Six patients experienced acute transplant rejection that required intensification of immunosuppressive therapy. Hematologic diagnosis included 12 monomorphic PTLD (10 diffuse large B lymphoma, 1 peripheral T cell lymphoma, 1 anaplastic large cell lymphoma), 2 classical Hodgkin lymphoma-type PTLD, 1 lymphoplasmacytic lymphoma and 1 extranodal marginal zone lymphoma. Histologic diagnosis was made on lymph node and on extranodal tissue in seven and nine cases, respectively. Available histologic analysis of tumor tissue demonstrated that EBV was positive in 62% of cases. CD20 expression was available in all but two patients and was positive in 71% of cases. At diagnosis seven patients were in stage IV, two in stage III, one in stage II and 6 in stage I; median IPI and Mayo prognostic score were 2 and 1, respectively. Ten patients presented with extranodal disease; central nervous system (CNS) involvement was not observed. Eleven patients received immuno-chemotherapy (including anthracyclines) with rituximab and two only chemotherapy; among them only four patients required reduction of doses because of liver/renal toxicity, and one patient experienced infective toxicity (WHO grade 1-2). One patient was treated with only radiotherapy and two patients were observed because of indolent disease. After a median follow up of 51 months (range 1-128) from the end of therapy, 13 patients are alive and in complete remission, two are lost to follow up and one patient died for progressive disease.CONCLUSIONS: Although the limited number of patients involved, and the peculiar subset of renal and liver allograft in which the risk of PTLD is low-intemediate, our study demonstrated that the majority of our patients developed aggressive NHL and an immuno-chemoterapic regimen is feasible, well tolerated and allow to obtain an elevated percentage of remission.
Settore MED/15 - Malattie del Sangue
European Hematology Association
http://www.haematologica.org/content/96/supplement_2/1.full.pdf+html
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