Extramedullary Plasmacytoma-like PTLD
Most monomorphic posttransplantation lymphoproliferative disorders (PTLDs) resemble diffuse large B-cell lymphoma or Burkitt lymphoma. Rare cases of PTLD resembling extramedullary plasmacytomas have also been described. This report describes the clinical, histologic, phenotypic, and genotypic findings in 4 cases of plasmacytoma-like PTLD (2 nodal, 1 adenoidal, and 1 cutaneous) and compares the findings with extramedullary involvement by plasma cell neoplasms arising in immunocompetent patients. Plasmacytoma-like PTLDs characteristically arise late after transplantation (mean, 7.0 years), show a variable association with Epstein-Barr virus (2/4 cases positive), and demonstrate histologic and phenotypic findings that overlap with immunocompetent extramedullary plasma cell neoplasms. None of the patients with plasmacytoma-like PTLD developed lytic bone lesions, and 3 of 4 patients had complete responses (>2 years) to reduction of immunosuppression, confirming the role of immunosuppression in the pathogenesis of these lesions. This report, which represents the first case series of plasmacytoma-like PTLD, clarifies the features of this rare subtype of PTLD.
Posttransplantation lymphoproliferative disorders (PTLDs) are a diverse group of lymphoid proliferations that arise secondary to immunosuppression in the setting of solid organ or bone marrow transplantation. Three primary categories are defined by the World Health Organization: "early lesions" composed of reactive plasmacytic hyperplasia and infectious mononucleosis–like proliferations, polymorphic PTLD, and monomorphic PTLD. The majority of PTLD cases are positive for Epstein-Barr virus (EBV), although EBV-negative PTLDs are increasingly recognized and may display distinct clinicopathologic features.
Most monomorphic PTLDs may be further classified as diffuse large B-cell lymphoma or Burkitt lymphoma. However, rare cases of monomorphic PTLDs resembling extramedullary plasmacytomas have also been reported. To date, the clinical and pathologic features of plasmacytoma-like PTLDs have been incompletely characterized. In this report, we describe the clinical, histologic, phenotypic, and genotypic findings in 4 cases of plasmacytoma-like PTLD and compare the findings with those seen in extramedullary involvement by plasma cell neoplasms (PCNs) arising in immunocompetent patients.
Abstract and Introduction
Abstract
Most monomorphic posttransplantation lymphoproliferative disorders (PTLDs) resemble diffuse large B-cell lymphoma or Burkitt lymphoma. Rare cases of PTLD resembling extramedullary plasmacytomas have also been described. This report describes the clinical, histologic, phenotypic, and genotypic findings in 4 cases of plasmacytoma-like PTLD (2 nodal, 1 adenoidal, and 1 cutaneous) and compares the findings with extramedullary involvement by plasma cell neoplasms arising in immunocompetent patients. Plasmacytoma-like PTLDs characteristically arise late after transplantation (mean, 7.0 years), show a variable association with Epstein-Barr virus (2/4 cases positive), and demonstrate histologic and phenotypic findings that overlap with immunocompetent extramedullary plasma cell neoplasms. None of the patients with plasmacytoma-like PTLD developed lytic bone lesions, and 3 of 4 patients had complete responses (>2 years) to reduction of immunosuppression, confirming the role of immunosuppression in the pathogenesis of these lesions. This report, which represents the first case series of plasmacytoma-like PTLD, clarifies the features of this rare subtype of PTLD.
Introduction
Posttransplantation lymphoproliferative disorders (PTLDs) are a diverse group of lymphoid proliferations that arise secondary to immunosuppression in the setting of solid organ or bone marrow transplantation. Three primary categories are defined by the World Health Organization: "early lesions" composed of reactive plasmacytic hyperplasia and infectious mononucleosis–like proliferations, polymorphic PTLD, and monomorphic PTLD. The majority of PTLD cases are positive for Epstein-Barr virus (EBV), although EBV-negative PTLDs are increasingly recognized and may display distinct clinicopathologic features.
Most monomorphic PTLDs may be further classified as diffuse large B-cell lymphoma or Burkitt lymphoma. However, rare cases of monomorphic PTLDs resembling extramedullary plasmacytomas have also been reported. To date, the clinical and pathologic features of plasmacytoma-like PTLDs have been incompletely characterized. In this report, we describe the clinical, histologic, phenotypic, and genotypic findings in 4 cases of plasmacytoma-like PTLD and compare the findings with those seen in extramedullary involvement by plasma cell neoplasms (PCNs) arising in immunocompetent patients.