Published online Oct 6, 2023. doi: 10.12998/wjcc.v11.i28.6889
Peer-review started: June 19, 2023
First decision: August 16, 2023
Revised: August 30, 2023
Accepted: September 6, 2023
Article in press: September 6, 2023
Published online: October 6, 2023
Processing time: 98 Days and 5.5 Hours
Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) differs from systemic anaplastic large cell lymphoma (sALCL) in cell biological behavior, clinical features, treatment, and outcome. PC-ALCL has been reported to rarely transition into sALCL, but the underlying mechanism is not clear. Here we report such a case with certain characteristics that shed light on this.
Herein, we report a 43-year-old male with symptoms of a skin nodule and histologically confirmed PC-ALCL with high expression of Ki-67. After three months of observation, two skin nodules re-appeared with muscle layer involvement and was histologically confirmed as sALCL. Seventeen months after receiving six cycles of CHOP regimen, the patient had pain in the chest and back, cough, shortness of breath, and night sweats. This was confirmed as relapse of sALCL by immunohistochemistry and several organs, such as the lung were involved as shown by positron emission tomography/computed tomography. After four cycles of DICE plus chidamide regimens followed by auto-hematopoietic stem cell transplantation (ASCT), complete remission (CR) duration was achieved for twelve months while the patient was on maintenance with chidamide (20 mg) pills.
This case had significantly high expression of Ki-67 when diagnosed as PC-ALCL initially and then transitioned into sALCL, which is rare. Auto-ASCT combined with demethylation drugs effectively maintained CR and prolonged progression free survival.
Core Tip: Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a primary cutaneous lymphoma and differs from systemic anaplastic large cell lymphoma (sALCL), which is a peripheral T cell lymphoma. There is no direct connection between them. This is the first report of the medical management, including auto-hematopoietic stem cell transplantation (ASCT) of a 43-year-old male patient who transitioned from the cutaneous to the systemic form of ALCL in Asia, and the second to be reported in world (the first being a Bulgarian treated with Brentuximab vedontin). We found high expression of Ki-67 in this case, for the first time, which may have played a role in the transition. This is also the first instance of application of auto-ASCT followed by maintenance with oral chidamide. In the future, an increasing number of patients will probably receive this therapy, and therein lies the significance of this case report.