Post-transplantation lymphoproliferative disorders: diagnosis, prognosis, and current approaches to therapy.
Post-transplantation lymphoproliferative disorders (PTLD) are a heterogenous group of abnormal lymphoid proliferations that occur after solid organ transplant (SOT) or hematopoietic transplantation. PTLDs consist of a disease spectrum ranging from hyperplasia to aggressive lymphomas with 60-70% being Epstein-Barr virus positive. The majority of cases are B-cell, although 10-15% are of T-cell origin or rarely Hodgkin lymphoma. Recent SOT series suggest PTLD occurs at a median of 36-40 months after transplant. Clinically, extra-nodal disease is common (up to 75-85%) including CNS involvement, which is seen in 10-15% of all cases. Since the first report over 40 years ago, PTLD has remained one of the most morbid complications associated with SOT. However, recent data suggests improved survival in the modern era, especially with the integration of early rituximab-based therapy. These studies utilized first line rituximab (+/- chemotherapy) together with reduced immune suppression (RI) for monomorphic and polymorphic PTLD. It will be critical in future studies to determine which PTLDs are most amenable to initial therapy with RI alone, versus RI/rituximab, versus RI/rituximab/chemotherapy. Additionally, novel therapeutics, such as adoptive immunotherapy, should continue to be explored.
Northwestern University Feinberg School of Medicine, Chicago, IL, USA. a-evens@northwestern.edu
Andrew M
AM
Evens
Rupali
R
Roy
Danielle
D
Sterrenberg
Michelle Z
MZ
Moll
Amy
A
Chadburn
Leo I
LI
Gordon
D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D018450:Disease Progression; D018572:Disease-Free Survival; D004334:Drug Administration Schedule; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000628:therapy; D004854:Herpesvirus 4, Human; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D016219:Immunotherapy, Adoptive; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology / Q000276:immunology / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D009017:Morbidity; D016377:Organ Transplantation / Q000009:adverse effects; D012307:Risk Factors; D000069283:Rituximab; D015996:Survival Rate; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology; D016378:Tissue Transplantation / Q000009:adverse effects; D016896:Treatment Outcome
D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab