Posttransplant Lymphoproliferative Disorders (PTLD)

Overview

Posttransplant lymphoproliferative disorders (PTLD) are a heterogeneous group of lymphoid or plasmacytic proliferations arising in immunosuppressed solid-organ or hematopoietic stem-cell transplant recipients. The majority are associated with Epstein-Barr virus (EBV) reactivation. PTLD ranges from polyclonal reactive proliferations to monomorphic B-cell (DLBCL-like), T-cell, or NK-cell lymphomas. Prognosis and treatment depend on histologic subtype, EBV status, and extent of immunosuppression reduction.

Cohorts in the corpus

  • mixed_pipseq_2017 — PIPseq pediatric pan-cancer cohort (Columbia University Medical Center), which includes PTLD cases among 101 high-risk pediatric patients PMID:28007021.

Recurrent alterations

  • PIPseq cohort: PTLD cases were included in the hematologic disease category (n=36 hematologic patients total); potentially actionable alterations were identified in 47% of hematologic cases overall PMID:28007021.

Subtypes

  • Non-destructive PTLD (plasmacytic hyperplasia, infectious mononucleosis-like, florid follicular hyperplasia).
  • Polymorphic PTLD.
  • Monomorphic PTLD (B-cell, T/NK-cell).
  • Classical Hodgkin lymphoma-type PTLD.

Therapeutic landscape

  • Reduction of immunosuppression is first-line; rituximab (anti-CD20) for EBV+ B-cell PTLD; chemotherapy for refractory disease.

Sources

  • PMID:28007021 — Oberg et al. PIPseq pediatric pan-cancer sequencing program (n=101).

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