Published online Jun 16, 2023. doi: 10.12998/wjcc.v11.i17.4072
Peer-review started: February 6, 2023
First decision: April 26, 2023
Revised: May 9, 2023
Accepted: May 16, 2023
Article in press: May 16, 2023
Published online: June 16, 2023
Processing time: 126 Days and 1.7 Hours
Angioimmunoblastic T-cell lymphoma (AITL), a unique subtype of peripheral T-cell lymphoma, has relatively poor outcomes. High-dose chemotherapy with autologous stem cell transplantation (ASCT) can achieve complete remission and improve outcomes. Unfortunately, subsequent T-cell lymphoma-triggered hemophagocytic lymphohistiocytosis (HLH) has a worse prognosis than B-cell lymphoma-triggered HLH.
We here report a 50-year-old woman with AITL who achieved a favorable outcome after developing HLH 2 mo after receiving high-dose chemotherapy/ ASCT. The patient was initially admitted to our hospital because of multiple enlarged lymph nodes. The final pathologic diagnosis, made on biopsy of a left axillary lymph node was AITL (Stage IV, Group A). Four cycles of the following chemotherapy regimen were administered: Cyclophosphamide 1.3 g, doxorubicin 86 mg, and vincristine 2 mg on day 1; prednisone 100 mg on days 1-5; and lenalidomide 25 mg on days 1-14. The interval between each cycle was 21 d. The patient received a conditioning regimen (busulfan, cyclophosphamide, and etoposide) followed by peripheral blood stem cell infusion. Unfortunately, she developed sustained fever and a low platelet count 17 d after ACST, leading to a diagnosis of HLH after ASCT. During treatment, she experienced thrombocytopenia and Pneumocystis carinii pneumonia. The patient was successfully treated with etoposide and glucocorticoids.
It is possible that development of HLH is related to immune reconstitution after ASCT.
Core Tip: Angioimmunoblastic T cell lymphoma (AITL), a subtype of mature peripheral T cell lymphoma, is characterized by intense inflammatory and immune reactions. Although high-dose chemotherapy with autologous stem cell transplantation (ASCT) is currently considered the optimal treatment option for AITL, subsequent development of hemophagocytic lymphohistiocytosis (HLH), a rare, life-threatening immunological syndrome, may still result in poor outcomes. In the present case, we speculate that HLH was likely attributable to immune reconstitution after ASCT. Successful immune reconstitution after ASCT is essential to improving overall survival and preventing opportunistic infections in patients with AITL.