ʻO ka lymphoma B-cell nui e hoʻolaha ʻia (DLBCL), ʻano kikowaena non-germinal, e pili ana i ka lua ihu a me nā sinuses-02

I Malaki 2021, ua hōʻike mai kekahi maʻi kāne he 52 makahiki mai ka Hikina ʻĀkau o Kina me ka puʻu ihu i ʻike ʻia i ka wā o ka nānā maʻamau. Ua ʻike ʻo ia i nā hōʻailona o ka ihu paʻa, ka ʻeha poʻo, ka ʻike pōwehiwehi, a me ka hou ʻana i ka pō, me ka ʻole o ke kuni a i ʻole ka pohō kaumaha.

Ua hōʻike nā hoʻokolohua mua i kahi nui o nā ʻiʻo palupalu e pili ana i ka lua ihu ʻākau a me nā sinuses, e hoʻopilikia ana i nā ʻano koʻikoʻi e like me ka orbit, ke kumu poʻo mua, ka sinus sphenoid, a me ka sinus ethmoid hema ma ka MRI. Ua hōʻike ka hoʻokolohua pathological o ka sinus maxillary ʻākau i ka lymphoma B-cell nui diffuse (DLBCL), ʻano kikowaena non-germinal.

Ua hōʻike ʻo Immunohistochemistry (IHC) i ka invasiveness kiʻekiʻe me ka hōʻike pālua ʻana o Ki-67 (90%+), CD20 (+), c-Myc (>80%+), Bcl-2 (>90%), Bcl-6 (+), CD10 (-), Mum1 (+), CD79a (+), CD30 (-), a me CyclinD1 (-), me ka ʻike ʻole ʻia o ka Epstein-Barr virus-encoded small RNA (EBER).

Ua ʻike ka Fluorescence in situ hybridization (FISH) i nā translocations Bcl-6 a me c-myc, akā ʻaʻohe translocation gene Bcl-2. Ua hōʻoia ka hoʻonohonoho hanauna hou (NGS) i nā mutations ma nā genes MYD88, CD79B, IGH-MYC, BAP1, a me TP53, e hōʻike ana i kahi lymphoma B-cell kiʻekiʻe me nā translocations MYC a me BCL2 a/a i ʻole BCL6.

Ua hōʻike ka Positron emission tomography-computed tomography (PET-CT) i nā ʻano ʻiʻo palupalu maʻamau ʻole ma ka lua ihu ʻākau a me ka sinus luna, ma kahi o 6.3x3.8cm ka nui, me nā palena ʻike ʻole ʻia. Ua piʻi aʻe ka ʻeha i luna i loko o ka sinus ethmoid ʻākau, i waho i ka paia medial o ka orbit a me ka ʻāpana intraorbital, a ma hope o ka sinus sphenoid a me ke kumu poʻo. Ua hōʻike ka ʻeha i ka hoʻonui ʻia o ka fluorodeoxyglucose (FDG) uptake me kahi SUVmax o 20. Ua ʻike ʻia ka mānoanoa o ka mucosal ma ka ethmoid hema a me ka sinus luna, me ka metabolism FDG maʻamau.

Ua hana mua ʻia ka maʻi ma o R2-CHOP, R-ESHAP, BEAM+ASCT, a me ka radiotherapy kūloko, me ka ʻike ʻia ʻana o ka holomua o ka maʻi. Ma muli o ke kūʻē ʻana o ka chemotherapy a me ka hoʻopili nui ʻana o nā ʻano he nui (me nā māmā, ke akepaʻa, ka spleen, a me nā iwi), ua ʻike ʻia ka maʻi me ka DLBCL refractory mua. Ua holomua koke ka maʻi me ka invasiveness kiʻekiʻe, nā pae LDH kiʻekiʻe, kahi helu International Prognostic Index (NCCN-IPI) i hoʻololi ʻia o 5, ka mutation TP53, a me ka subtype MCD, e ʻike ana i ka relapse i loko o 6 mau mahina ma hope o ka hoʻoili ʻana o ka autologous.

Ma hope o ka hoʻōla ʻana i ka bridging, ua loaʻa i ka mea maʻi ka lāʻau steroid no kekahi manawa me ka pane maikaʻi ʻole. ʻO ka lāʻau ma hope mai ua komo pū me nā antibodies monoclonal CD79 i hui pū ʻia me bendamustine a me mechlorethamine hydrochloride, e hopena ana i ka emi nui ʻana o nā pae LDH a me ka emi ʻana o ka puʻupuʻu i ʻike ʻia.

Ma hope o ka hoʻomākaukau kūleʻa ʻana o ka lāʻau lapaʻau CAR-T, ua hana ʻia ka chemotherapy lymphocyte depletion (lymphodepletion) me ka regimen FC, e hoʻokō ana i ka hoʻomaʻemaʻe lymphocyte i manaʻo ʻia a me ka leukopenia koʻikoʻi ma hope. Eia nō naʻe, ʻekolu lā ma mua o ka hoʻokomo ʻana o CAR-T, ua loaʻa i ka mea maʻi ke kuni, herpes zoster ma ka ʻāpana lumbar, a me nā pae serum lactate dehydrogenase (LDH) kiʻekiʻe a hiki i ka 25.74ng/ml, e hōʻike ana i kahi hanana ʻino (AE) o ka maʻi ikaika i hui pū ʻia. Ke noʻonoʻo nei i ka hoʻonui ʻia o ka pilikia o ka hoʻokomo ʻana o CAR-T ma muli o ka maʻi ikaika, hiki ke alakaʻi i nā hopena make, ua loaʻa i ka mea maʻi nā antibiotics ākea e uhi ana i nā pathogens like ʻole.

Ma hope o ka hoʻokomo ʻia ʻana o CAR-T, ua loaʻa i ka mea maʻi ke kuni kiʻekiʻe i ka lā hoʻokomo, e holomua ana i ka dyspnea, hemoptysis, a me ka hoʻonui ʻia ʻana o nā hōʻailona pulmonary i ka lā ʻekolu. Ua hōʻike ka pulmonary venous CT angiography i ka lā ʻelima i nā opacities ground-glass i hoʻopuehu ʻia a me nā loli interstitial, e hōʻoia ana i ke kahe koko pulmonary. ʻOiai ka pale mua ʻana i nā steroids ma muli o ka hiki ke kāohi ʻia ʻo CAR-T, a me ka mālama kākoʻo i kālele ʻia i ka hoʻokele anti-infection, ua hōʻike ka maʻi o ka mea maʻi i ka hoʻomaikaʻi palena ʻia.

I ka lā ʻehiku, ua ʻike ʻia ka hoʻonui nui ʻana o ka helu kope gene CAR i loko o ke koko peripheral, e koi ana i kahi hoʻoponopono lapaʻau me ka methylprednisolone haʻahaʻa (40mg-80mg). ʻElima mau lā ma hope mai, ua emi iho nā rales māmā bilateral, a ua kāohi nui ʻia nā hōʻailona hemoptysis.

I ka lā ʻewalu, ua hōʻike ka lāʻau lapaʻau CAR-T i ka hopena kupaianaha. I loko o hoʻokahi mahina wale nō o ka lāʻau lapaʻau CAR-T, ua loaʻa i ka mea maʻi ke kala piha (CR). Ua hōʻoia nā hoʻokolohua ma hope a hiki i Iulai 2023 ua noho ka mea maʻi ma CR, e hōʻike ana i kahi pane hohonu i ka lāʻau lapaʻau CAR-T a me ka hiki ke ho'ōla ʻia.