Ukusasaza i-large B-cell lymphoma (DLBCL), uhlobo oluphakathi olungelulo olwe-germinal, olubandakanya umngxuma wempumlo kunye nee-sinuses-02

NgoMatshi 2021, isigulana esineminyaka engama-52 ubudala esivela kuMantla-mpuma weTshayina safumana isiqabu sempumlo esafunyanwa ngexesha lokuhlolwa rhoqo. Safumana iimpawu zokuxinana kwempumlo, iintloko ezibuhlungu, ukubona okufipheleyo, kunye nokubila ebusuku, ngaphandle komkhuhlane okanye ukwehla kobunzima bomzimba.

Uvavanyo lokuqala lubonise ubunzima obukhulu bezicubu ezithambileyo ezibandakanya umngxuma wempumlo yasekunene kunye neesinuses, nto leyo echaphazela izakhiwo ezibalulekileyo ezifana ne-orbit, isiseko se-anterior skull, i-sphenoid sinus, kunye ne-left ethmoid sinus kwi-MRI. Uvavanyo lwe-pathological lwe-right maxillary sinus lubonisa ukuba i-diffuse large B-cell lymphoma (DLBCL), uhlobo lwe-non-germinal center subtype.

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 (-), kunye ne-CyclinD1 (-), ngaphandle kwe-RNA encinci (EBER) enokubonwa yi-Epstein-Barr virus-encoded).

I-Fluorescence in situ hybridization (FISH) ifumanise ukudluliselwa kwe-Bcl-6 kunye ne-c-myc, kodwa akukho ukudluliselwa kwe-Bcl-2 gene. I-Next-generation sequencing (NGS) iqinisekisile utshintsho kwi-MYD88, CD79B, IGH-MYC, BAP1, kunye ne-TP53 genes, nto leyo ebonisa i-B-cell lymphoma ephezulu ene-MYC kunye ne-BCL2 kunye/okanye ukudluliselwa kwe-BCL6.

I-Positron emission tomography-computed tomography (PET-CT) ibonise izihlunu ezithambileyo ezingalinganiyo kwimpumlo yasekunene nakwi-sinus ephezulu, malunga ne-6.3x3.8cm ngobukhulu, kunye nemida engacacanga. Isilonda sanda saya phezulu kwi-sinus ye-ethmoid yasekunene, ngaphandle ukuya eludongeni oluphakathi lwe-orbit kunye nommandla we-intraorbital, kwaye ngasemva ukuya kwi-sinus ye-sphenoid kunye nesiseko se-skull. Isilonda sabonisa ukwanda kokuthathwa kwe-fluorodeoxyglucose (FDG) nge-SUVmax engama-20. Ukuqina kwe-mucosal kwaphawulwa kwi-sinus ye-ethmoid yasekhohlo kunye ne-sinus ephezulu, kunye ne-metabolism eqhelekileyo ye-FDG.

Isigulana sasikhe safumana i-R2-CHOP, i-R-ESHAP, i-BEAM+ASCT, kunye ne-radiotherapy yasekuhlaleni, kwaye kwabonwa ukuqhubela phambili kwesi sifo. Ngenxa yokuxhathisa i-chemotherapy kunye nokubandakanyeka okukhulu kwamalungu amaninzi (kubandakanya imiphunga, isibindi, i-spleen, kunye namathambo), isigulana safunyaniswa sine-DLBCL yokuqala engalunganga. Esi sifo saqhubeka ngokukhawuleza ngokungenelela okuphezulu, amanqanaba aphezulu e-LDH, amanqaku ama-5 e-International Prognostic Index (NCCN-IPI), i-TP53 mutation, kunye ne-MCD subtype, ephinda ibuyele kwi-relaxation kwiinyanga ezi-6 emva kokufakelwa kwe-autologous.

Emva konyango oludibeneyo, isigulana safumana unyango lwe-steroid okwethutyana kodwa impendulo yaso yancinci. Unyango lwamva nje lwaluquka ii-CD79 monoclonal antibodies ezidityaniswe ne-bendamustine kunye ne-mechlorethamine hydrochloride, nto leyo eyabangela ukwehla okukhulu kwamanqanaba e-LDH kunye nokuncipha okubonakalayo kwethumba.

Emva kokulungiselela ngempumelelo unyango lwe-CAR-T, isigulana senziwa i-chemotherapy yokuphelelwa yi-lymphocyte (lymphodepletion) nge-FC regimen, safumana i-lymphocyte clearance eyayicetywayo kunye ne-leukopenia enzima eyalandelayo. Nangona kunjalo, kwiintsuku ezintathu ngaphambi kokufakwa kwe-CAR-T, isigulana saba nomkhuhlane, i-herpes zoster kwindawo ye-lumbar, kunye namanqanaba aphezulu e-serum lactate dehydrogenase (LDH) ukuya kuthi ga kwi-25.74ng/ml, okubonisa ukuba kunokwenzeka ukuba kubekho isiganeko esibi sosulelo oluxubeneyo (AE). Ukuqwalasela umngcipheko ophezulu wokufakwa kwe-CAR-T ngenxa yosulelo olusebenzayo, olunokubangela iziphumo ezibulalayo, isigulana safumana ii-antibiotics ezibanzi ezigubungela iintsholongwane ezahlukeneyo.

Emva kokufakwa kwe-CAR-T, isigulana saba nomkhuhlane ophezulu ngosuku lokufakwa kwe-infusion, saya ekuphelelweni yintliziyo, saza saya kwi-hemoptysis, saza saya sibi kakhulu iimpawu zemiphunga ngosuku lwesithathu. I-Pulmonary venous CT angiography ngosuku lwesihlanu yabonisa ukungabonakali kakuhle kweglasi esemhlabeni kunye notshintsho oluphakathi kwemithambo yegazi, okuqinisekisa ukopha kwemithambo yegazi emiphungeni. Nangona kwakungasasetyenziswa ii-steroids ngenxa yokucinezelwa kwe-CAR-T, kunye nonyango oluxhasayo olugxile kulawulo lokulwa nosulelo, imeko yesigulana yabonisa ukuphucuka okuncinci.

Ngomhla wesixhenxe, kwafunyaniswa ukwanda okukhulu kwenani lekopi ye-CAR gene egazini elingaphandle, nto leyo eyabangela ukuba kulungiswe unyango nge-methylprednisolone yedosi ephantsi (40mg-80mg). Emva kweentsuku ezintlanu, i-bilateral lung rales yehla, kwaye iimpawu ze-hemoptysis zalawulwa ngokuphawulekayo.

Ngomhla wesibhozo, unyango lwe-CAR-T lubonakalise ukusebenza kakuhle. Kwinyanga enye nje yonyango lwe-CAR-T, isigulana sifumene uxolelo olupheleleyo (CR). Uvavanyo olulandelayo ukuya kuthi ga ngoJulayi 2023 luqinisekisile ukuba isigulana sihlala kwi-CR, nto leyo ebonisa impendulo enzulu kunyango lwe-CAR-T kunye nokuba sinokunyangwa.