Kuparadzira hombe B-cell lymphoma (DLBCL), isiri-germinal centre subtype, inosanganisira mhino ne sinuses-02

Muna Kurume 2021, mumwe murume ane makore makumi mashanu nemaviri ekuberekwa kubva kuchamhembe kwakadziva kumabvazuva kweChina akaunzwa nemhino yakawanikwa panguva yekuongororwa. Aiva nezviratidzo zvemhino, kutemwa nemusoro, kusaona zvakanaka, uye dikita usiku, asingafivha kana kuonda.

Ongororo dzekutanga dzakaratidza hunyoro hwakapfava hunosanganisira mhango yemhino yekurudyi uye sinuses, zvichikanganisa zvimiro zvakakomba senge orbit, anterior skull base, sphenoid sinus, uye kuruboshwe ethmoid sinus paMRI. Pathological kuongororwa kwekurudyi maxillary sinus yakaratidza kupararira hombe B-cell lymphoma (DLBCL), isiri-germinal centre subtype.

Immunohistochemistry (IHC) yakaratidza kupindira kwepamusoro nekutaura kaviri kweKi-67 (90%+), CD20 (+), c-Myc (>80%+), Bcl-2 (>90%), Bcl-6 (+) , CD10 (-), Mum1 (+), CD79a (+), CD30 (-), uye CyclinD1 (-), isina detectable Epstein-Barr virus-encoded small RNA (EBER).

Fluorescence in situ hybridization (FISH) yaonekwa Bcl-6 uye c-myc translocations, asi hapana Bcl-2 gene translocation. Next-generation sequencing (NGS) yakasimbisa shanduko muMYD88, CD79B, IGH-MYC, BAP1, uye TP53 genes, zvichiratidza high-grade B-cell lymphoma ine MYC uye BCL2 uye / kana BCL6 translocations.

Positron emission tomography-computed tomography (PET-CT) yakaratidza masaisa ematishu akapfava asina kujairika mumhino yekurudyi uye sinus yepamusoro, inenge 6.3x3.8cm muhukuru, ine miganhu isina kujeka. Chironda chacho chaikwira chichienda kumusoro mukati mekurudyi ethmoid sinus, kunze kumadziro epakati pe orbit uye intraorbital region, uye kumashure kune sphenoid sinus uye skull base. Chirwere chacho chakaratidza kuwedzera kwefluorodeoxyglucose (FDG) kutorwa neSUVmax ye20. Mucosal thickening yakaonekwa mu ethmoid yekuruboshwe uye sinus yepamusoro, ine yakajairika FDG metabolism.

Murwere akambosangana neR2-CHOP, R-ESHAP, BEAM + ASCT, uye radiotherapy yemunharaunda, nekufambira mberi kwechirwere. Nekuda kwekurwisa chemotherapy uye kubatanidzwa kwakanyanya kwenhengo dzakawanda (kusanganisira mapapu, chiropa, spleen, uye mapfupa), murwere akaonekwa aine yekutanga refractory DLBCL. Chirwere ichi chakafambira mberi nekukurumidza nepamusoro-soro, mazinga eLDH akakwidziridzwa, akagadziridzwa International Prognostic Index (NCCN-IPI) chikamu che5, TP53 mutation, uye MCD subtype, inosangana nekudzoka zvakare mukati memwedzi ye6 mushure mekugadzirisa autologous.

Kutevera bhiriji kurapwa, murwere akagamuchira kwenguva pfupi kurapwa kwesteroid nekusapindura. Gare gare kurapwa kwaisanganisira maCD79 monoclonal antibodies akasanganiswa nebendamustine uye mechlorethamine hydrochloride, zvichiita kuti kuderedzwa kukuru kwemazinga eLDH uye kuoneka kwebundu shrinkage.

Mushure mekubudirira kwekugadzirira kweCAR-T kurapwa, murwere akawana lymphocyte depletion (lymphodepletion) chemotherapy ne FC regimen, achiwana chinangwa che lymphocyte clearance uye yakatevera leukopenia yakaoma. Zvisinei, mazuva matatu kusati kwaitwa CAR-T infusion, murwere akaita fivha, herpes zoster munharaunda ye lumbar, uye yakakwirira serum lactate dehydrogenase (LDH) mazinga kusvika 25.74ng / ml, zvichiratidza kuti inogona kuitika yakasanganiswa-mhando yehutachiona hunokanganisa chiitiko chakaipa (AE). ) Tichifunga nezve njodzi yakawedzera yekuiswa kweCAR-T nekuda kwehutachiona hunoshanda, hunogona kutungamirira kumigumisiro inouraya, murwere akagamuchira maantibioti e-broad-spectrum anovhara zvirwere zvakasiyana-siyana.

Kutevera kuisirwa kweCAR-T, murwere akatanga kupisa kwakanyanya pazuva rekuisirwa, zvichienda kudyspnea, hemoptysis, uye kuwedzera kwezviratidzo zvemapapu nezuva retatu. Pulmonary venous CT angiography pazuva rechishanu yakaratidza yakapararira pasi-girazi opacities uye interstitial shanduko, zvichisimbisa pulmonary hemorrhage. Pasinei nekutanga kudziviswa kwe steroid nekuda kwekukwanisa kudzvinyirirwa kweCAR-T, uye kurapa kwekutsigira kwakanangana nekugadzirisa hutachiona hwehutachiona, mamiriro emurwere akaratidza zvishoma zvishoma.

Pazuva rechinomwe, kukosha kweCAR gene kopi nhamba yekuwedzera yakaonekwa muropa reperipheral, zvichiita kuti kugadziriswa kwekurapa ne-low-dose methylprednisolone (40mg-80mg). Mazuva mashanu gare gare, mapapu emapapu maviri akadzikira, uye zviratidzo zvehemoptysis zvakadzorwa zvakanyanya.

Pakazosvika zuva resere, kurapwa kweCAR-T kwakaratidza kushanda kunoshamisa. Mukati memwedzi mumwe chete wekurapwa kweCAR-T, murwere akawana kuregererwa kwakakwana (CR). Ongororo dzakazotevera kusvika muna Chikunguru 2023 dzakasimbisa kuti murwere akaramba ari muCR, zvichiratidza mhinduro yakadzama kuCAR-T kurapwa uye mukana wekurapa.