| | Case no. | Tx year | Sex/age | Organ | Underlying disease | EBV serostatus | Time from transplant to | Peak titer of EBV load (copies/mL) | Median titer of EBV load (copies/mL) | Involved PTLD sites/primary site | Histologic diagnosis (pathologic findings) | Treatment (rituximab†, RIS, chemotherapy‡) | Clinical outcome (PTLD, graft loss/function) | Alive at 1 year | | EBV DNAemia | PTLD diagnosis |
| | 1 | 2019 | 21/M | Kidney | Nephronophthisis | D+/R− | 4 months | Early
4 months | 796 | 229 | Bilateral palatine and adenoid tonsils; cervical lymph nodes; and spleen | Early lesions (infectious mononucleosis-like)
+EBER | RIS | Complete response | Yes |
| | 2 | 2017 | 64/F | Liver | Primary biliary cirrhosis | D+/R+ | N/A | Early
3 months | N/A | N/A | Liver allograft; porta hepatis, portocaval, retrocaval, and mesenteric lymph nodes | Polymorphic or monomorphic, unclear which subtype
−EBER | RIS
Rituximab | Complete response | Yes |
| | 3 | 2016 | 63/M | Liver | Alcoholic/HCV cirrhosis | D+/R+ | N/A | Late
23 months | N/A | N/A | Liver allograft; posterolateral spleen; and retrocrural lymph nodes | Monomorphic/Burkitt’s lymphoma
EBER | RIS
Rituximab chemo | Complete response | Yes |
| | 4 | 2016 | 58/M | Liver | Alcoholic cirrhosis | D+/R− | 3 months | Early
7 months | 1,964,383 | 16,112 | Liver allograft; adrenal gland; axillary and mediastinal LAD | Polymorphic
+EBER | RIS
Rituximab | Complete response | Yes |
| | 5 | 2015 | 53/M | Liver | NASH cirrhosis; HCC | D+/R+ | N/A | Late
32 months | N/A | N/A | Cervical, axillary, mediastinal, R hilar lymph nodes; multiple subpleural pulm nodules; and liver allograft | Monomorphic/diffuse large B-cell lymphoma
−EBER | RIS
Rituximab chemo | Complete response | Yes |
| | 6 | 2015 | 68/M | Kidney | Diabetes mellitus type 2 | D+/R− | 8 months | Early
9 months | 124,693 | 4,597 | Submandibular mass; lung; and upper abdominal/pancreatic mass | Monomorphic/diffuse large B-cell lymphoma
+EBER | RIS
Rituximab chemo | Partial response | No, death at 11 months |
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Tx, transplant; EBV, Epstein–Barr virus; PTLD, posttransplant lymphoproliferative disease; D, donor; R, recipient; N/A, not applicable; EBER, Epstein–Barr virus-encoded small RNA; RIS, reduction in immunosuppression; chemo, chemotherapy; pulm, pulmonary. †Rituximab regimens included induction therapy (4 doses administered weekly) followed by consolidative therapy (3-4 doses administered every 3 weeks). ‡Chemotherapy regimens for these three patients included (case no. 3) etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (DA-EPOCH); (case no. 5) rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP); (case no. 6) R-CHOP and later EPOCH with bortezomib.
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