Multidisciplinary treatment of NK/T-cell lymphoma associated hemophagocytic lymphohistiocytosis
This article presents the multidisciplinary team(MDT)approach to the diagnosis and treatment of a case involving nasal extranodal NK/T-cell lymphoma(ENKTCL)complicated with hemophagocytic lymphohisti-ocytosis(HLH).The patient,admitted with persistent fever and refractory to standard antimicrobials,exhibited pancytopenia,a nasal mass,and evidence of EBV infection.By the time of presentation at our facility,the patient had progressed to the late stages of HLH.Despite strong clinical indicators suggestive of primary nasal ENK TCL,the patient's hemodynamic instability,coupled with coagulopathy and severe thrombocytopenia,posed an exceedingly high risk for a nasopharyngeal biopsy to ascertain the etiology of secondary HLH.Following compre-hensive deliberation by our MDT,it was determined that the existing evidence was inconclusive to distinguish be-tween EBV-HLH and ENKTCL.Consequently,the decision was made to administer the DEP regimen to manage HLH,with the intention of conducting a pathological biopsy once the patient's condition was stabilized.His-topathological examination of the nasal biopsy confirmed the diagnosis of ENKTCL(nasal type).After the two-week course of DEP therapy,the patient experienced a relapse of symptoms,indicating that the HLH was refrac-tory to the treatment.In light of the histopathological findings,the therapeutic strategy was revised to the L-MEP regimen,which successfully induced remission of HLH.Thereafter,the patient was promptly initiated on P-Ge-mOx chemotherapy targeting the underlying ENKTCL.The patient was discharged following clinical remission.This case exemplifies the pivotal role of MDT in managing aggressive conditions such as ENKTCL-HLH.Fur-thermore,the successful resolution of refractory lymphoma associated HLH with the L-MEP regimen implies the therapeutic potential of mitoxantrone liposomal in combination with etoposide and methylprednisolone for adult HLH,contributing to the evidence for future clinical trials.
hemophagocytic lymphohistiocytosisextranodal natural killer/T-cell lymphomaL-MEP regi-menmultidisciplinary team
NETL
NSTL
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