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Post-transplant Lymphoproliferative Disorders (PTLD)

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❓What❓

  • PTLD refers to a spectrum of lymphoproliferative disorders (lymphoid and/or plasmacytic proliferations) stemming from post-transplantation immunosuppression

❓Epidemiology❓

  • ~2/3 = EBV ⊕: post-transplantation immunosuppression is felt to result in ↓ control of EBV-immortalized B-cells, which can derive from either the donor/recipient, & which often develop within the first year
  • ~1/3 = EBV ⊖: etiology not fully elucidated, but may be related to EBV infections no longer detectable vs. other unidentified viruses driving chronic antigenic stimulation. EBV ⊖ cases generally present much later (~5 years post-transplantation) & carry much worse survival (median ~1 month)
  • Solid organ: most common malignancy complicating SOT, & degree of immunosuppression for survival of transplanted organ correlates with PTLD risk (e.g. heart > kidney). Stem cell: accounts for only a minority of secondary cancers, but mortality is high if present
  • Risk factors: degree of immunosuppression (i.e. early transplantation period = ↑ risk [e.g. >80% within first year]), EBV D+/R– (i.e. recipient is likely to receive/develop an EBV infection early in postoperative period)

❓Presentation❓

🌙 Recipient-derived → Multi-system ⊕ Allograft-directed

  • Constitutional symptoms & reticuloendothelial activation (e.g. HSM, LAD)
  • Lymphoma’s laboratory signature (e.g. cytopenias, tumor lysis syndrome, monoclonal protein, ↑ ALP)
  • Extranodal infiltrative disease resulting in organ dysfunction vs. compression (~1/4 with CNS lesions, ~1/4 with allograft lesions, but notably other sites more common such as GI infiltration)

🌙 Donor-derived → Allograft-directed

  • Same as above, but clinical, lab, & imaging signature likely less striking due to allograft-limited disease (i.e. not multi-system)
PTLD arising at the renal pelvis resulting in hydronephrosis & post-renal allograft dysfunction
PTLD arising at the renal pelvis resulting in hydronephrosis & post-renal allograft dysfunction

❓Diagnosis❓

Suggestive (i.e. “screen”) 🚨

  • CBC with cytopenias
  • ↑ LDH, ↑ uric acid
  • ↑ ALP > TBili

Pre-Biopsy ✋

  • Stratify: EBV viral load (↑↑↑ = suggestive), PET-CT (staging)
  • Can’t Miss: blood cultures (routine, AFB, fungal) to assess for disseminated granulomatous infections +/- sputum cultures if pulmonary symptoms, or other directed infectious diseases testing if concerning symptoms

Confirmation = Biopsy 🔬

  • Excisional biopsy of affected tissue

📚 References 📚