Hodgkin Lymphoma-Like Posttransplant Lymphoproliferative Disorder (HL-Like PTLD) Simulates Monomorphic B-Cell PTLD Both Clinically and Pathologically

Abstract

Although Hodgkin lymphoma-like posttransplantation lymphoproliferative disorder (HL-like PTLD) has been grouped with classic Hodgkin lymphoma type PTLD (HL-PTLD), controversy remains as to whether it is truly a form of HL or whether it should be more appropriately classified as a form of B-cell PTLD. Because only few cases of HL-like PTLD have been reported, their pathologic nature and clinical behavior have not been well defined. This report characterized 5 cases of HL-like PTLD with respect to their immunophenotype, EBV status, clonality, and clinical outcome. All of the patients were male, with ages ranging from 1.5 to 55 years at diagnosis. PTLD developed from 4 months to 6 years following solid organ transplantation (3 hearts, 1 kidney, 1 liver), and involved both nodal and extranodal sites. All were EBV-related (EBER+) with the large neoplastic cells CD20/CD79a positive but CD15 negative. Immunoglobulin gene rearrangements were detected in 3 of 5 tested. All patients were managed by initial reduction/withdrawal of immunosuppression, with 2 also receiving chemotherapy for non-HL. Three patients died of progressive disease within 2 to 3 months after diagnosis, 1 is alive and well 2 years later, and the fifth was disease free but died of unrelated causes (graft coronary disease) 2 years later. We conclude that, although HL-like PTLD morphologically simulates classic HL PTLD, there are important immunophenotypic, molecular genetic, and clinical differences, suggesting it is in fact most often a B-cell PTLD. Distinction between HL and HL-like PTLD may be important for clinical management and prognosis.

Using Epstein-Barr Viral Load Assays To Diagnose, Monitor, and Prevent Posttransplant Lymphoproliferative Disorder

“…Rare cases of classical Hodgkin lymphoma-type PTLD occur late after transplant (beyond the first year) and invariably contain EBV within the malignant Reed-Sternberg/Hodgkin cells (148). The immunophenotype is that of nodular sclerosis or mixed cellularity Hodgkin lymphoma, and response to therapy is generally favorable (174).…”

Post-transplant lymphoproliferative disorders

“…Limited observations of the clinical outcome suggested that HLlike PTLD is an aggressive disorder like a true B-cell type PTLD. 35 This is a very rare disease and it is difficult to collect evidence that would unequivocally demonstrate that such cases should be distinguished from CHL-type PTLD. However, it was suggested that CHL-type PTLD may respond best to chemotherapies often used in CHL, whereas an HL-like PTLD may be more amenable to combinations of chemo-immunotherapy often used for NHL.…”

Hepatic involvement by lymphoproliferative disorders post liver transplantation: PTLD.Int. Survey

“…A total of 21 international published studies [13,[20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39] were found that met our criteria. Table 1 summarizes data of the enrolled studies.…”