Sabalalisa i-B-cell lymphoma enkulu (i-DLBCL), uhlobo oluncane lwesikhungo olungezona amagciwane, olubandakanya ikhala kanye nezono-02

NgoMashi 2021, isiguli sowesilisa esineminyaka engu-52 ubudala saseNyakatho-mpumalanga yeChina sethula amakhala atholwa ngesikhathi sokuhlolwa okujwayelekile. Waba nezimpawu zokuminyana emakhaleni, ephathwa yikhanda, angaboni kahle, ajuluke ebusuku, angabi namfiva noma anciphe.

Ukuhlola kokuqala kwembula inqwaba yezicubu ezithambile ezihlanganisa ikhala elingakwesokudla kanye nezono, ezithinta izakhiwo ezibucayi njenge-orbit, isisekelo sogebhezi lwangaphambili, i-sphenoid sinus, kanye ne-ethmoid sinus yesokunxele ku-MRI. Ukuhlolwa kwe-pathological kwe-maxillary sinus engakwesokudla kwaphakamisa ukuthi kusakazeke i-B-cell lymphoma enkulu (i-DLBCL), uhlobo oluncane lwesikhungo esingewona amagciwane.

I-Immunohistochemistry (IHC) ibonise ukuhlasela okuphezulu ngokuveza okukabili kwe-Ki-67 (90%+), CD20 (+), c-Myc (>80%+), Bcl-2 (>90%), Bcl-6 (+) , CD10 (-), Mum1 (+), CD79a (+), CD30 (-), ne-CyclinD1 (-), engenayo i-RNA encane efakwe ikhodi ye-Epstein-Barr (EBER).

I-Fluorescence in situ hybridization (FISH) ithole ukudluliselwa kwe-Bcl-6 ne-c-myc, kodwa akukho ukudluliselwa kofuzo lwe-Bcl-2. Ukulandelana kwesizukulwane esilandelayo (NGS) kuqinisekise ukuguqulwa kofuzo ku-MYD88, CD79B, IGH-MYC, BAP1, kanye ne-TP53, okubonisa izinga eliphezulu le-B-cell lymphoma ene-MYC ne-BCL2 kanye/noma nokudluliselwa kwe-BCL6.

I-Positron emission tomography-computed tomography (PET-CT) ibonise izicubu ezithambile ezingajwayelekile endaweni yamakhala engakwesokudla kanye ne-sinus ephakeme, ecishe ibe ngu-6.3x3.8cm ngosayizi, enemingcele engacacile. Isilonda sinwetshwa sikhuphukele phezulu kusinus ethmoid esingakwesokudla, ngaphandle siye obondeni olumaphakathi lwe-orbit kanye nesifunda se-intraorbital, futhi nangemuva ku-sphenoid sinus nesisekelo sogebhezi. Isilonda sibonise ukwanda kwe-fluorodeoxyglucose (FDG) nge-SUVmax engu-20. Ukuqina kwe-mucosal kwaphawulwa ku-ethmoid yesokunxele kanye ne-sinus ephakeme, ne-FDG metabolism evamile.

Isiguli phambilini sasike sathola i-R2-CHOP, i-R-ESHAP, i-BEAM+ASCT, kanye ne-radiotherapy yasendaweni, kwaphawulwa ukuqhubeka kwesifo. Ngenxa yokumelana nokwelashwa ngamakhemikhali kanye nokubandakanyeka okukhulu kwezitho eziningi (kuhlanganise namaphaphu, isibindi, ubende, namathambo), isiguli satholakala sine-DLBCL ephikisayo eyinhloko. Lesi sifo sathuthuka ngokushesha ngokuhlasela okuphezulu, amazinga e-LDH aphakeme, amaphuzu aguquliwe we-International Prognostic Index (NCCN-IPI) we-5, TP53 mutation, kanye ne-MCD subtype, ebhekana nokuphindaphinda phakathi kwezinyanga ezingu-6 emva kokufakelwa kwe-autologous.

Ngemva kokwelashwa kwe-bridging, isiguli sathola ukwelashwa kwe-steroid kafushane ngokungaphenduli kahle. Ukwelashwa kwakamuva kwakuhlanganisa amasosha omzimba e-CD79 monoclonal ahlanganiswe ne-bendamustine ne-mechlorethamine hydrochloride, okuholele ekwehleni okukhulu kwamazinga e-LDH kanye nokuncipha kwesimila okuphawulekayo.

Ngemuva kokulungiswa ngempumelelo kokwelashwa kwe-CAR-T, isiguli sathola ukwelashwa ngamakhemikhali kwe-lymphocyte (i-lymphodepletion) ngohlelo lwe-FC, kwafinyelela imvume ye-lymphocyte ehlosiwe kanye ne-leukopenia enzima eyalandela. Kodwa-ke, ezinsukwini ezintathu ngaphambi kokufakwa kwe-CAR-T, isiguli saba nemfiva, i-herpes zoster esifundeni se-lumbar, kanye namazinga aphezulu e-serum lactate dehydrogenase (LDH) aze afike ku-25.74ng/ml, okubonisa isenzakalo esibi esingase sibe khona sohlobo oluxubile olusebenzayo (AE). ). Uma kucatshangelwa ukwanda kwengozi yokumnika i-CAR-T ngenxa yokutheleleka okusebenzayo, okungase kuholele emiphumeleni ebulalayo, isiguli sathola ama-antibiotic abanzi ahlanganisa amagciwane ahlukahlukene.

Ngemva kokumpontshelwa i-CAR-T, isiguli saba nemfiva enkulu ngosuku lokumnika, kwaqhubekela ku-dyspnea, ukopha, kanye nezimpawu zamaphaphu eziba zimbi ngosuku lwesithathu. I-CT angiography ye-pulmonary venous ngosuku lwesihlanu yembule ukukhanya kwengilazi yaphansi ehlakazekile kanye nezinguquko ze-interstitial, okuqinisekisa ukopha kwamaphaphu. Naphezu kokugwema kokuqala kwe-steroids ngenxa yokucindezelwa okungenzeka kwe-CAR-T, kanye nokwelashwa okusekelayo okugxile ekulawuleni ukulwa nokutheleleka, isimo sesiguli sabonisa ukuthuthukiswa okulinganiselwe.

Ngosuku lwesikhombisa, ukunwetshwa kwenombolo yekhophi yekhophi yofuzo lwe-CAR ebalulekile kwatholwa egazini le-peripheral, okwenze ukuba kulungiswe ukwelashwa ngedosi ephansi ye-methylprednisolone (40mg-80mg). Ezinsukwini ezinhlanu kamuva, amaphaphu ezinhlangothi zombili ancipha, futhi izimpawu ze-hemoptysis zalawulwa ngokuphawulekayo.

Ngosuku lwesishiyagalombili, ukwelashwa kwe-CAR-T kwabonisa ukusebenza kahle okuphawulekayo. Phakathi nenyanga eyodwa yokwelashwa kwe-CAR-T, isiguli sazuza ukukhululwa okuphelele (CR). Ukuhlolwa okwalandela kwaze kwaba nguJulayi 2023 kwaqinisekisa ukuthi isiguli sahlala ku-CR, okubonisa ukusabela okujulile ekwelashweni kwe-CAR-T kanye namandla okwelapha.