Haploidentical hematopoietic stem cell transplantation for pediatric patients with chronic active Epstein–Barr virus infection: a retrospective analysis of a single center

Massive pericardial effusion due to chronic active Epstein–Barr virus infection successfully treated with PD-1 blockade: A case report

“…[9] A recent retrospective study in China also demonstrated a favorable 3-year OS rate of 92.0% for patients with CAEBV who received the L-DEP regimen (pegylated-asparaginase, liposomal doxorubicin, etoposide, and methylprednisolone) prior to planned haploidentical HSCT. [13] In addition, JAK inhibitors can also achieve long-term stable remission and provide an opportunity for allo-HSCT. [11] However, cardiac complications in CAEBV patients are beyond cure, and 78% die of disease progression or transplantation-related events.…”

Allogeneic hematopoietic stem cell transplantation with the modified myeloablative conditioning regimen for children with chronic active Epstein–Barr virus infection

“…All patients were treated with 1−4 courses of the L‐DEP regimen to reduce/eliminate EBV‐infected T/NK cells and suppress the disease activity after they were diagnosed with CAEBV: liposomal doxorubicin 25 mg·m −2 on day 1; etoposide 100 mg·m −2 on the first day of each week. PEG‐asparaginase 2000 U·m −2 on day 5; methylprednisolone 15 mg·kg −1 ·d −1 on days 1−3, 2 mg·kg −1 ·d −1 on days 4−7 and 1 mg·kg −1 ·d −1 on days 8−14 followed by gradual tapering the following week 12,13 . Seven patients, whose initial pathologies were highly suspected of tumorigenesis or whose disease remained active or who had a high burden of residual lesions after 1−3 courses of L‐DEP therapy, received ESCAP regimen: etoposide 150 mg·m −2 on day 1; cytosine arabinoside 1.5 mg·m −2 twice/d ×8 times starting from the night of day 1; L‐asparaginase 6000 U·m −2 once every other day ×5 times starting from day 5; methylprednisolone 2.5 mg·kg −1 twice/d ×8 times starting from the night of day 1, 2 mg·kg −1 ·d −1 on days 6−12, 1 mg·kg −1 ·d −1 on days 13−15, 0.5 mg·kg −1 ·d −1 on days 16−18, 0.25 mg·kg −1 ·d −1 on days 19−21 5,13 .…”

“… 5 , 13 Before the initiation of conditioning, two patients with active disease received L‐DEC regimen: low‐dose etoposide 30 mg·m −2 ·d −1 and cytosine arabinoside 20 mg·m −2 ·d −1 were continuously administered for 24 h for 0.5−2 weeks. 5 , 13 …”

“…PEG‐asparaginase 2000 U·m −2 on day 5; methylprednisolone 15 mg·kg −1 ·d −1 on days 1−3, 2 mg·kg −1 ·d −1 on days 4−7 and 1 mg·kg −1 ·d −1 on days 8−14 followed by gradual tapering the following week 12,13 . Seven patients, whose initial pathologies were highly suspected of tumorigenesis or whose disease remained active or who had a high burden of residual lesions after 1−3 courses of L‐DEP therapy, received ESCAP regimen: etoposide 150 mg·m −2 on day 1; cytosine arabinoside 1.5 mg·m −2 twice/d ×8 times starting from the night of day 1; L‐asparaginase 6000 U·m −2 once every other day ×5 times starting from day 5; methylprednisolone 2.5 mg·kg −1 twice/d ×8 times starting from the night of day 1, 2 mg·kg −1 ·d −1 on days 6−12, 1 mg·kg −1 ·d −1 on days 13−15, 0.5 mg·kg −1 ·d −1 on days 16−18, 0.25 mg·kg −1 ·d −1 on days 19−21 5,13 . Before the initiation of conditioning, two patients with active disease received L‐DEC regimen: low‐dose etoposide 30 mg·m −2 ·d −1 and cytosine arabinoside 20 mg·m −2 ·d −1 were continuously administered for 24 h for 0.5−2 weeks 5,13 …”

“…5,13 Before the initiation of conditioning, two patients with active disease received L-DEC regimen: low-dose etoposide 30 mg⋅m −2 ⋅d −1 and cytosine arabinoside 20 mg⋅m −2 ⋅d −1 were continuously administered for 24 h for 0.5−2 weeks. 5,13 Transplantation Evaluation of CAEBV disease state 12 : the disease state prior to allo-HSCT was assessed according to clinical characteristics and EBV load before being divided into active disease (AD), partial remission (PR), and complete remission (CR). AD: persistent inflammatory manifestations, such as fever, lymphadenectasis, hepatosplenomegaly, pancytopenia, progressive skin damage, and persistent positive plasma EBV-DNA.…”