Ukusabalalisa i-large B-cell lymphoma (DLBCL), uhlobo lwesikhungo esingeyona i-germinal, oluhilela umgodi wekhala kanye nama-sinuses-02

NgoMashi 2021, isiguli sesilisa esineminyaka engu-52 ubudala esivela eNyakatho-mpumalanga yeShayina sathola ukuhlushwa kwamakhala ngesikhathi sokuhlolwa okuvamile. Sathola izimpawu zokuminyana kwamakhala, ikhanda elibuhlungu, ukubona okufiphele, kanye nokujuluka ebusuku, ngaphandle komkhuhlane noma ukwehla kwesisindo.

Ukuhlolwa kokuqala kwembule inqwaba yezicubu ezithambile ezibanzi ezihilela umgodi wempumulo wangakwesokudla kanye nama-sinuses, okuthinta izakhiwo ezibalulekile njenge-orbit, isisekelo sekhanda elingaphambili, i-sphenoid sinus, kanye ne-left ethmoid sinus ku-MRI. Ukuhlolwa kwe-pathological kwe-right maxillary sinus kusikisele i-diffuse large B-cell lymphoma (DLBCL), uhlobo lwesikhungo esingeyona i-germinal.

I-Immunohistochemistry (IHC) ibonise ukuhlasela okuphezulu okune-Ki-67 (90%+), i-CD20 (+), i-c-Myc (>80%+), i-Bcl-2 (>90%), i-Bcl-6 (+), i-CD10 (-), i-Mum1 (+), i-CD79a (+), i-CD30 (-), kanye ne-CyclinD1 (-), ngaphandle kwe-RNA encane (EBER) efakwe igciwane le-Epstein-Barr.

I-Fluorescence in situ hybridization (FISH) ithole ukudluliselwa kwe-Bcl-6 kanye ne-c-myc, kodwa akukho ukudluliselwa kwe-Bcl-2 gene. Ukulandelana kwesizukulwane esilandelayo (NGS) kuqinisekisile izinguquko ku-MYD88, CD79B, IGH-MYC, BAP1, kanye ne-TP53 genes, okubonisa i-B-cell lymphoma yezinga eliphezulu ene-MYC kanye ne-BCL2 kanye/noma ukudluliselwa kwe-BCL6.

I-Positron emission tomography-computed tomography (PET-CT) ibonise izinqwaba zezicubu ezithambile ezingajwayelekile emgodini wempumulo kwesokudla kanye ne-sinus ephezulu, cishe engu-6.3x3.8cm ngobukhulu, enemingcele engaqondakali. Inxeba landa laya phezulu e-sinus ye-ethmoid yesokudla, ngaphandle odongeni oluphakathi lwe-orbit kanye nesifunda sangaphakathi kwe-orbital, kanye nangemuva kwe-sinus ye-sphenoid kanye nesisekelo sekhanda. Inxeba libonise ukwanda kokuthathwa kwe-fluorodeoxyglucose (FDG) nge-SUVmax engu-20. Ukuqina kwe-mucosal kwaphawulwa e-sinus yesobunxele ye-ethmoid kanye ne-sinus ephezulu, kanye ne-metabolism evamile ye-FDG.

Isiguli sasike sathola ukwelashwa ngemisebe ye-R2-CHOP, i-R-ESHAP, i-BEAM+ASCT, kanye nokwelashwa ngemisebe yendawo, kwabonakala ukuqhubeka kwesifo. Ngenxa yokumelana namakhemikhali kanye nokubandakanyeka okukhulu kwezitho eziningi (kufaka phakathi amaphaphu, isibindi, ubende, namathambo), isiguli satholakala sine-DLBCL eyinhloko engaphikisi. Lesi sifo saqhubeka ngokushesha ngokuhlasela okuphezulu, amazinga aphezulu e-LDH, amaphuzu angu-5 e-International Prognostic Index (NCCN-IPI) aguquliwe, i-TP53 mutation, kanye ne-MCD subtype, siphinde sibuyele esimeni saso ezinyangeni eziyi-6 ngemuva kokufakelwa kwe-autologous.

Ngemva kokwelashwa okuhlanganisiwe, isiguli sathola ukwelashwa kwe-steroid okwesikhashana kodwa impendulo yaso yangaba yinhle. Ukwelashwa kamuva kwakuhlanganisa ama-CD79 monoclonal antibodies ahlanganiswe ne-bendamustine kanye ne-mechlorethamine hydrochloride, okwaholela ekunciphiseni okukhulu kwamazinga e-LDH kanye nokuncipha kwesimila okubonakalayo.

Ngemva kokulungiselela ngempumelelo ukwelashwa kwe-CAR-T, isiguli senziwe i-chemotherapy yokuncipha kwe-lymphocyte (i-lymphodepletion) ngohlelo lwe-FC, safinyelela ekususweni kwe-lymphocyte okuhlosiwe kanye ne-leukopenia enzima eyalandela. Kodwa-ke, ezinsukwini ezintathu ngaphambi kokufakwa kwe-CAR-T, isiguli saba nomkhuhlane, i-herpes zoster esifundeni se-lumbar, kanye namazinga aphezulu e-serum lactate dehydrogenase (LDH) afinyelela ku-25.74ng/ml, okubonisa ukuthi kungenzeka kube nesenzakalo esibi sokutheleleka okusebenzayo (i-AE). Uma sibheka ingozi eyengeziwe yokufakwa kwe-CAR-T ngenxa yokutheleleka okusebenzayo, okungenzeka kuholele emiphumeleni ebulalayo, isiguli sathola ama-antibiotic e-broad-spectrum amboza amagciwane ahlukahlukene.

Ngemva kokufakwa kwe-CAR-T, isiguli saba nomkhuhlane omkhulu ngosuku lokufakwa, saqhubekela ekuphefumuleni, ekuqhekekeni kwegazi, kanye nokwanda kwezimpawu zamaphaphu ngosuku lwesithathu. I-pulmonary venous CT angiography ngosuku lwesihlanu yembula ukuvuleka kwengilazi engaphansi komhlaba okuhlakazekile kanye nezinguquko zangaphakathi, okuqinisekisa ukuphuma kwegazi emaphaphu. Naphezu kokugwema kokuqala ama-steroid ngenxa yokucindezelwa kwe-CAR-T okungenzeka, kanye nokwelashwa okusekelayo okugxile ekuphathweni kokulwa nokutheleleka, isimo sesiguli sabonisa ukuthuthuka okuncane.

Ngosuku lwesikhombisa, kwatholakala ukwanda okukhulu kwenani lekhophi yezakhi zofuzo ze-CAR egazini elingaphandle, okwaholela ekulungisweni kokwelashwa nge-methylprednisolone yomthamo ophansi (40mg-80mg). Ezinsukwini ezinhlanu kamuva, ukusabalala kwamaphaphu amabili kwehla, futhi izimpawu ze-hemoptysis zalawulwa ngokuphawulekayo.

Ngosuku lwesishiyagalombili, ukwelashwa kwe-CAR-T kwabonisa ukusebenza kahle okumangalisayo. Phakathi nenyanga eyodwa yokwelashwa kwe-CAR-T, isiguli sathola ukuphulukiswa okuphelele (CR). Ukuhlolwa okulandelayo kwaze kwaba nguJulayi 2023 kwaqinisekisa ukuthi isiguli sisese-CR, okubonisa impendulo ejulile ekwelashweni kwe-CAR-T kanye nethuba lokwelashwa.