Kupararira kweB-cell lymphoma yakakura (DLBCL), rudzi rwepakati rusiri rwegerminal, runosanganisira mhino ne sinuses-02

MunaKurume 2021, murwere wechirume ane makore makumi mashanu nemaviri ekuberekwa wekuNortheast China akaonekwa aine mhino yakawandisa yakawanikwa panguva yekuongororwa kwaakaitwa nguva dzose. Akasangana nezviratidzo zvekuvharika kwemhino, musoro, kuona zvisipo, uye kudikitira husiku, asina fivha kana kudzikira uremu.

Ongororo dzekutanga dzakaratidza kuti pane nyama yakapfava yakawanda ine mhino yekurudyi ne sinuses, zvichikanganisa zvivako zvakakosha zvakaita se orbit, anterior skull base, sphenoid sinus, uye left ethmoid sinus paMRI. Kuongororwa kwe pathological kwe right maxillary sinus kwakaratidza diffuse large B-cell lymphoma (DLBCL), iyo isiri germinal center subtype.

Immunohistochemistry (IHC) yakaratidza kuvapo kwakanyanya kwehutachiona nekutaura kaviri kweKi-67 (90%+), CD20 (+), c-Myc (>80%+), Bcl-2 (>90%), Bcl-6 (+), CD10 (-), Mum1 (+), CD79a (+), CD30 (-), uye CyclinD1 (-), isina RNA diki (EBER) inoonekwa nehutachiona hweEpstein-Barr.

Kusanganiswa kweFluorescence in situ (FISH) kwakaona kuchinjika kweBcl-6 nec-myc, asi hapana kuchinjika kweBcl-2 gene. Kutevedzana kwechizvarwa chinotevera (NGS) kwakasimbisa shanduko mumajini eMYD88, CD79B, IGH-MYC, BAP1, uye TP53, zvichiratidza kuti pane lymphoma yeB-cell yepamusoro ine MYC neBCL2 uye/kana kuchinjika kweBCL6.

Positron emission tomography-computed tomography (PET-CT) yakaratidza matishu akapfava asina kurongeka mumhino yekurudyi uye sinus yepamusoro, saizi inosvika 6.3x3.8cm, ine miganhu isingaonekwe. Chironda ichi chakakwira kusvika mu sinus yekurudyi ye ethmoid, kunze kusvika kumadziro epakati pe orbit nenzvimbo yemukati meorbital, uye kumashure kusvika ku sinus yesphenoid nebase yedehenya. Chironda ichi chakaratidza kuwedzera kwe fluorodeoxyglucose (FDG) uptake neSUVmax ye20. Kukora kwemucosal kwakaonekwa mu sinus yekuruboshwe ye ethmoid ne superior, neFDG metabolism yakajairika.

Murwere akamboitwa R2-CHOP, R-ESHAP, BEAM+ASCT, uye radiotherapy yemuno, uye kufambira mberi kwechirwere kwakaonekwa. Nekuda kwekusagona kurapwa nechemotherapy uye kupindira kwakanyanya kwenhengo dzakawanda (kusanganisira mapapu, chiropa, chiropa, nemapfupa), murwere akaonekwa aine DLBCL yekutanga isingashande. Chirwere ichi chakafambira mberi nekukurumidza nekupindira kwakanyanya, mazinga eLDH akakwira, chibodzwa cheInternational Prognostic Index (NCCN-IPI) che5, TP53 mutation, uye MCD subtype, zvichidzokera shure mukati memwedzi mitanhatu mushure mekuisirwa autologous.

Mushure mekurapwa kwemushonga wekubatanidza masero, murwere akapihwa mushonga we steroid kwenguva pfupi asi mhinduro yake haina kunaka. Kurapwa kwakazotevera kwaisanganisira maCD79 monoclonal antibodies akasanganiswa nebendamustine nemechlorethamine hydrochloride, zvikaita kuti huwandu hweLDH huderere zvakanyanya uye bundu ridzikire zvakanyanya.

Mushure mekugadzirira zvakanaka kurapwa kweCAR-T, murwere akarapwa chemotherapy yekudzikira kwemasero eropa (lymphodepletion) neFC regimen, zvikaita kuti lymphocyte clearance ibude uye leukopenia yakanyanya. Zvisinei, mazuva matatu CAR-T isati yaiswa, murwere akatanga fivha, herpes zoster mu lumbar region, uye mazinga e serum lactate dehydrogenase (LDH) akakwira kusvika pa25.74ng/ml, zvichiratidza mukana wekuti pave nechirwere chakasanganiswa (AE). Tichifunga nezvekuwedzera kwenjodzi yeCAR-T inokonzerwa nehutachiona, izvo zvinogona kukonzera mhedzisiro inouraya, murwere akapihwa mishonga inorwisa mabhakitiriya yakawanda inofukidza hutachiona hwakasiyana-siyana.

Mushure mekupihwa CAR-T, murwere akatanga kupisa muviri zvakanyanya pazuva rekupihwa, zvichikonzera kushaya simba, kubuda ropa mumuviri, uye zviratidzo zvemapapu zvichiwedzera kuipa pazuva rechitatu. Kuongororwa kweCT angiography yemapapu pazuva rechishanu kwakaratidza kupararira kwemagirazi epasi uye shanduko dzemukati memapapu, zvichiratidza kubuda ropa mumapapu. Pasinei nekudziviswa kwekutanga kwemishonga yesteroid nekuda kwekudzvinyirirwa kweCAR-T, uye kurapwa kwakanangana nekurapa kwekudzivirira hutachiona, mamiriro emurwere akaratidza kuvandudzika kushoma.

Pazuva rechinomwe, kuwedzera kukuru kwenhamba yemagene eCAR kwakaonekwa muropa rekunze, zvikaita kuti pave nekuchinja kwekurapwa ne methylprednisolone ine dose shoma (40mg-80mg). Mazuva mashanu gare gare, kupararira kwemapapu maviri kwakaderera, uye zviratidzo zve hemoptysis zvakadzorwa zvakanyanya.

Pazuva rechisere, kurapwa kweCAR-T kwakaratidza kushanda zvakanaka. Mukati memwedzi mumwe chete wekurapwa kweCAR-T, murwere akawana relieve yakakwana (CR). Ongororo dzakatevera kusvika muna Chikunguru 2023 dzakaratidza kuti murwere akaramba ari muCR, zvichiratidza kuti akagamuchira CAR-T therapy zvakadzama uye kuti anogona kurapwa.