How I treat posttransplant lymphoproliferative disorders

Abstract

Posttransplant lymphoproliferative disorder (PTLD) is a potentially fatal disorder arising after solid organ transplant (SOT) or hematopoietic stem cell transplant (HSCT). Iatrogenically impaired immune surveillance and Epstein-Barr virus (EBV) primary infection/reactivation are key factors in the pathogenesis. However, current knowledge on all aspects of PTLD is limited due to its rarity, morphologic heterogeneity, and the lack of prospective trials. Furthermore, the broad spectrum of underlying immune disorders and the type of graft represent important confounding factors. Despite these limitations, several reviews have been written aimed at offering a guide for pathologists and clinicians in diagnosing and treating PTLD. Rather than providing another classical review on PTLD, this “How I Treat” article, based on 2 case reports, focuses on specific challenges, different perspectives, and novel insights regarding the pathogenesis, diagnosis, and treatment of PTLD. These challenges include the wide variety of PTLD presentation (making treatment optimization difficult), the impact of EBV on pathogenesis and clinical behavior, and the controversial treatment of Burkitt lymphoma (BL)-PTLD.

Post-transplant lymphoproliferative disorder presenting with skin ulceration in a renal transplant recipient who achieved sustained remission with rituximab therapy: A case report

“…The risk factors for developing PTLD include EBV serostatus (higher risk of developing PTLD when an EBV seronegative organ recipient receives an organ from a seropositive donor), type of organ transplant, intensity of immunosuppression and age: There is a higher incidence of PTLD among extrarenal transplant recipients (5-7); the incidence of PTLD is highest with haploidentical hematopoietic stem cell transplant (HSCT), heart/lung and multivisceral transplants (≤20%), followed by liver (4.5%), combination heart-lung (2.5%), pancreas (2%), kidney (1-1.5%) and matched related and unrelated HSCT (0.5-1%) (7,8); in addition, pediatric patients are at greater risk compared with adults (5).…”

Untitled

“…The allograft and the gastrointestinal tract are frequently involved; however, PTLD may occur at any site, including the skin and the central nervous system. 3 Epstein-Barr virus and posttransplant lymphoproliferative disease Seventy percent of PTLDs are associated with infection or reactivation of EBV. Healthy children exhibit a strong immune response against EBV, mainly by cytotoxic T cells, resolving the infection.…”

“…3 Early-type PTLDs are nondestructive lymphoplasmacytic proliferations. Polymorphic PTLDs are the most challenging.…”

Enfermedad linfoproliferativa en el injerto renal. Reporte de un caso y revisión de la literatura

“…Las alteraciones linfoproliferativas se clasifican en hiperplasia benigna, donde hay proliferación difusa de células mononucleares con tejido conservado; PTLD tipo polimórfico, caracterizado por linfocitos en distintas fases de diferenciación, daño tisular y zonas de necrosis; tipo monomórfico, donde el tejido tiene transformación neoplásica; y, por último, el linfoma de Hodgkin clásico 7,9,10 . El PTLD que surge de las células B se identifica con la tinción CD20.…”

“…El PTLD que surge de las células B se identifica con la tinción CD20. La reducción de la inmunosupresión es la primera línea de tratamiento en PTLD polimórfico temprano 9 .…”