Ukusasaza i-B-cell lymphoma enkulu (i-DLBCL), i-non-germinal center subtype, ebandakanya umngxuma weempumlo kunye ne-sinuses-02

NgoMatshi ka-2021, isigulana esineminyaka engama-52 ubudala esivela kuMntla-mpuma we-China sibonisa ubunzima beempumlo obufunyenwe ngexesha lovavanyo lwesiqhelo. Waba neempawu zokuxinana kweempumlo, intloko ebuhlungu, ukubona luzizi, nokubila ebusuku, ngaphandle komkhuhlane okanye ukubhitya.

Iimviwo zokuqala zibonise ubunzima obuninzi bezicubu ezithambileyo ezibandakanya umgodi wempumlo wasekunene kunye nezono, ezichaphazela izakhiwo ezibalulekileyo ezifana ne-orbit, isiseko seskull sangaphambili, i-sphenoid sinus, kunye ne-ethmoid sinus ekhohlo kwi-MRI. Uvavanyo lwe-Pathological sinus ye-maxillary sinus lucebise ukuba i-B-cell lymphoma enkulu (i-DLBCL), i-non-germinal center subtype.

I-Immunohistochemistry (IHC) ibonise ukuhlaselwa okuphezulu kunye nokubonakaliswa kabini kwe-Ki-67 (90%+), CD20 (+), c-Myc (>80%+), Bcl-2 (>90%), Bcl-6 (+) , CD10 (-), Mum1 (+), CD79a (+), CD30 (-), kunye ne-CyclinD1 (-), ngaphandle kokubonwa kwe-Epstein-Barr virus-encoded small RNA (EBER).

I-Fluorescence in situ hybridization (I-FISH) ichonge ukutshintshwa kwe-Bcl-6 kunye ne-c-myc, kodwa akukho gene ye-Bcl-2 yokudluliselwa. Ulandelelwano lwesizukulwana esilandelayo (NGS) luqinisekisile utshintsho kwi-MYD88, CD79B, IGH-MYC, BAP1, kunye ne-TP53 yofuzo, ebonisa i-B-cell lymphoma ephezulu kunye ne-MYC kunye ne-BCL2 kunye / okanye i-BCL6 yokudluliselwa.

I-Positron emission tomography-computed tomography (PET-CT) ibonise ubuninzi bezicubu ezithambileyo ezingaqhelekanga kwindawo yempumlo yasekunene kunye ne-sinus ephezulu, malunga ne-6.3x3.8cm ngobukhulu, kunye nemida engabonakaliyo. Isilonda solulelwe phezulu kwi-ethmoid sinus yasekunene, ngaphandle ukuya kudonga oluphakathi lwe-orbit kunye nommandla we-intraorbital, kwaye ngasemva kwi-sphenoid sinus kunye nesiseko sokakayi. I-lesion ibonise ukunyuka kwe-fluorodeoxyglucose (FDG) kunye ne-SUVmax ye-20. Ukuqina kwe-mucosal kwaphawulwa kwi-ethmoid yasekhohlo kunye ne-sinus ephezulu, kunye ne-metabolism ye-FDG eqhelekileyo.

Isigulane sasikhe safumana i-R2-CHOP, i-R-ESHAP, i-BEAM+ ASCT, kunye ne-radiotherapy yendawo, kunye nokuqhubela phambili kwesifo. Ngenxa yokuxhathisa i-chemotherapy kunye nokubandakanyeka okubanzi kwamalungu amaninzi (kubandakanywa nemiphunga, isibindi, i-spleen, kunye namathambo), isigulane safunyaniswa ukuba sine-DLBCL ye-refractory yokuqala. Isifo saqhubela phambili ngokukhawuleza kunye nokuhlaselwa okuphezulu, amanqanaba aphezulu e-LDH, i-Modified International Prognostic Index (NCCN-IPI) amanqaku e-5, i-TP53 mutation, kunye ne-MCD subtype, ejongene nokuphindaphinda kwiinyanga ze-6 emva kokutshintshwa kwe-autologous.

Ukulandela unyango lwe-bridging, isigulana safumana ngokufutshane unyango lwe-steroid kunye nempendulo engalunganga. Unyango lwamva luquka i-CD79 monoclonal antibodies edityaniswe ne-bendamustine kunye ne-mechlorethamine hydrochloride, okukhokelela ekunciphiseni okubonakalayo kumanqanaba e-LDH kunye nokuncipha kwe-tumor ephawulekayo.

Emva kokulungiselela ngempumelelo unyango lwe-CAR-T, isigulane safumana i-lymphocyte (i-lymphodepletion) i-chemotherapy kunye ne-FC regimen, ukuphumeza ukucaciswa kwe-lymphocyte kunye ne-leukopenia enzima elandelayo. Nangona kunjalo, iintsuku ezintathu ngaphambi kokuba i-CAR-T infusion, isigulane sivelise umkhuhlane, i-herpes zoster kwingingqi ye-lumbar, kunye ne-serum lactate dehydrogenase ephakamileyo (LDH) ephakamileyo amanqanaba ukuya kwi-25.74ng / ml, ebonisa ukuba kunokwenzeka ukusuleleka kwintsholongwane esebenzayo (AE) ). Ukuqwalasela umngcipheko wokonyuka kwe-CAR-T ngenxa yosulelo olusebenzayo, olunokuthi lukhokelela kwiziphumo ezibulalayo, isigulane safumana i-antibiotics ebanzi egubungela iintsholongwane ezahlukeneyo.

Ukulandela ukufakwa kwe-CAR-T, isigulane saba nomkhuhlane ophezulu ngosuku lokuxilwa, ukuqhubela phambili kwi-dyspnea, i-hemoptysis, kunye neempawu ze-pulmonary ezibuhlungu ngosuku lwesithathu. I-CT angiography ye-pulmonary venous ngosuku lwesihlanu ibonakalise ukukhanya kweglasi esasazekileyo yomhlaba kunye notshintsho lwangaphakathi, oluqinisekisa ukopha kwemiphunga. Nangona ukuphepha kokuqala kwe-steroids ngenxa yokunyanzeliswa kwe-CAR-T, kunye nonyango oluxhasayo lugxininise ekulawuleni ukusuleleka kwintsholongwane, imeko yesigulane yabonisa ukuphuculwa okulinganiselwe.

Ngomhla wesixhenxe, i-CAR gene ikopi ebalulekileyo yokwandiswa kwenombolo yafunyanwa kwigazi le-peripheral, ikhuthaza ukulungiswa kwonyango nge-dose ephantsi ye-methylprednisolone (40mg-80mg). Kwiintsuku ezintlanu kamva, imiphunga yamazwe amabini yehla, kwaye iimpawu ze-hemoptysis zazilawulwa ngokuphawulekayo.

Ngomhla wesibhozo, unyango lwe-CAR-T lubonise ukusebenza okumangalisayo. Kwinyanga nje enye yonyango lwe-CAR-T, isigulane sifumene uxolelo olupheleleyo (CR). Iimviwo ezilandelayo ukuya kutsho ngoJulayi 2023 ziqinisekisile ukuba isigulane sihlala kwi-CR, ebonisa impendulo enzulu kunyango lwe-CAR-T kunye nokunyangwa.