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PRIMARY CENTRAL NERVOUS SYSTEM POST-TRANSPLANT LYMPHOPROLIFERATIVE DISORDER (PTLD-CNS) IS AN EBV-RELATED DISEASE WITH GOOD RESPONSE TO ISS REDUCTION AND RADIOTHERAPY: SINGLE-CENTER STUDY OF 16 CASES
Author(s): ,
Egyla M. Cavalcante
Affiliations:
UNIFESP - Federal University Of S?o Paulo,S?o Paulo,Brazil
,
Guilherme Fleury Perini
Affiliations:
UNIFESP - Federal University Of S?o Paulo,S?o Paulo,Brazil
,
Joao Garibaldi Jr
Affiliations:
UNIFESP - Federal University Of S?o Paulo,S?o Paulo,Brazil
,
Adriana M. D. Penna
Affiliations:
UNIFESP - Federal University Of S?o Paulo,S?o Paulo,Brazil
,
Juliano C. Vargas
Affiliations:
UNIFESP - Federal University Of S?o Paulo,S?o Paulo,Brazil
,
Jose Osmar M. Pestana
Affiliations:
Hospital do Rim e Hipertens?o,S?o Paulo,Brazil
Otavio C. G. Baiocchi
Affiliations:
UNIFESP - Federal University Of S?o Paulo,S?o Paulo,Brazil
(Abstract release date: 05/21/15) EHA Library. Cavalcante E. 06/12/15; 102963; PB1680 Disclosure(s): UNIFESP - Federal University Of S?o Paulo
Hematology - Clinical and Experimental Oncology
Dr. Egyla Cavalcante
Dr. Egyla Cavalcante
Contributions
Abstract
Abstract: PB1680

Type: Publication Only

Background

Post-transplant lymphoproliferative disorders are a heterogenous group of diseases occurring in the setting of post-transplant immunossupression. Clinically, extranodal involvement  is common, and it occurs in the CNS in approximately 7-15% of cases.  Most data on PTLD-CNS are based on case series/reports, with an exception of a multicenter study of 84 patients. In this group, PTLD-CNS showed to be a poor prognosis, late-ocurring EBV-associated disease. We retrospectively analyzed 16 cases of PTLD-CNS in Hospital do Rim, Sao Paulo, Brazil.



Aims
To analyze the cases of PTLD with CNS involvement diagnosed on our center over the past 17 years.

Methods

From 1998 to 2014, a total of 11,284 kidney transplants were performed at Hospital do Rim e Hipertensão/UNIFESP (Federal University of São Paulo). We retrospectively analyzed cases of PTLD with CNS involvement diagnosed over the past 17 years. Only confirmed cases of PTLD with available clinical and epidemiological data were included.



Results

From 1999 to 2014, a total of 20 patients were diagnosed with PTLD-CNS. Four patients were excluded from the analysis due to conflict data. Among 16 patients, the median age at time of diagnosis was 40, with a male:female ratio of 0,77:1. 56% of patients received ATG at the time of transplant. Regarding immunossupression (ISS), all patients received Prednisone, 37,5% received FK, 43,7% received MMF, and 56,2% received CSA. The median time of transplant to PTLD was 93 months, with 25% patients with polymorphic PTLD and 75% with monomorphic PTLD (DLBCL). Regarding EBV positivity, 14/16 were EBV+, with 1 EBV- and 1 inconclusive. All patients had ISS reduction, 43% of patients received brain radiotherapy. Only 1 patient received high dose Methotrexate. 75% of patients responded to treatment. No death related to lymphoma progression was observed: four patients died, all of them due to infectious complications, and none of them during therapy.



Summary

In our population, CNS-PTLD was an EBV-related disease with good response to ISS reduction and radiotherapy, with most patients achieving responses. No death due to disease progression was observed. Late effects of brain radiation, however, should be taken in account when deciding the best therapy for this rare complication of transplant.



Keyword(s): Post-transplant lymphoproliferative disorder, Transplant

Session topic: Publication Only
Abstract: PB1680

Type: Publication Only

Background

Post-transplant lymphoproliferative disorders are a heterogenous group of diseases occurring in the setting of post-transplant immunossupression. Clinically, extranodal involvement  is common, and it occurs in the CNS in approximately 7-15% of cases.  Most data on PTLD-CNS are based on case series/reports, with an exception of a multicenter study of 84 patients. In this group, PTLD-CNS showed to be a poor prognosis, late-ocurring EBV-associated disease. We retrospectively analyzed 16 cases of PTLD-CNS in Hospital do Rim, Sao Paulo, Brazil.



Aims
To analyze the cases of PTLD with CNS involvement diagnosed on our center over the past 17 years.

Methods

From 1998 to 2014, a total of 11,284 kidney transplants were performed at Hospital do Rim e Hipertensão/UNIFESP (Federal University of São Paulo). We retrospectively analyzed cases of PTLD with CNS involvement diagnosed over the past 17 years. Only confirmed cases of PTLD with available clinical and epidemiological data were included.



Results

From 1999 to 2014, a total of 20 patients were diagnosed with PTLD-CNS. Four patients were excluded from the analysis due to conflict data. Among 16 patients, the median age at time of diagnosis was 40, with a male:female ratio of 0,77:1. 56% of patients received ATG at the time of transplant. Regarding immunossupression (ISS), all patients received Prednisone, 37,5% received FK, 43,7% received MMF, and 56,2% received CSA. The median time of transplant to PTLD was 93 months, with 25% patients with polymorphic PTLD and 75% with monomorphic PTLD (DLBCL). Regarding EBV positivity, 14/16 were EBV+, with 1 EBV- and 1 inconclusive. All patients had ISS reduction, 43% of patients received brain radiotherapy. Only 1 patient received high dose Methotrexate. 75% of patients responded to treatment. No death related to lymphoma progression was observed: four patients died, all of them due to infectious complications, and none of them during therapy.



Summary

In our population, CNS-PTLD was an EBV-related disease with good response to ISS reduction and radiotherapy, with most patients achieving responses. No death due to disease progression was observed. Late effects of brain radiation, however, should be taken in account when deciding the best therapy for this rare complication of transplant.



Keyword(s): Post-transplant lymphoproliferative disorder, Transplant

Session topic: Publication Only

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