Hoʻolahalaha nui B-cell lymphoma (DLBCL), non-germinal center subtype, e pili ana i ka nasal cavity a me nā sinuses-02

I Malaki 2021, ua hōʻike ʻia kahi maʻi kāne 52 ona makahiki mai Northeast Kina me ka nui o ka ihu i ʻike ʻia i ka wā o ka nānā maʻamau. Ua ʻike ʻo ia i nā hōʻailona o ka nasal congestion, headaches, blurred vision, and night sweats, me ka ʻole o ke kuni a me ka pohō kaumaha.

Ua hōʻike ʻia nā hoʻokolohua mua i ka nui o ka ʻiʻo palupalu e pili ana i ka lua ihu ʻākau a me nā sinuses, e pili ana i nā hale koʻikoʻi e like me ka orbit, ke kumu iwi mua, ka sphenoid sinus, a me ka ethmoid sinus hema ma MRI. ʻO ka nānā ʻana i ka pathological o ka maxillary sinus akau i manaʻo ʻia e hoʻopuehu nui B-cell lymphoma (DLBCL), non-germinal center subtype.

Hōʻike ka Immunohistochemistry (IHC) i ka invasive kiʻekiʻe me ka ʻōlelo pālua 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 ʻo Epstein-Barr virus-encoded RNA liʻiliʻi (EBER).

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

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

Ua hana mua ka mea maʻi i ka R2-CHOP, R-ESHAP, BEAM + ASCT, a me ka radiotherapy kūloko, me ka ʻike ʻana i ka piʻi ʻana o ka maʻi. Ma muli o ka pale ʻana i ka chemotherapy a me ke komo ʻana o nā ʻōkana he nui (me nā māmā, ate, spleen, a me nā iwi), ua ʻike ʻia ka mea maʻi me ka DLBCL refractory mua. Ua holo wikiwiki ka maʻi me ka invasiveness kiʻekiʻe, nā pae LDH kiʻekiʻe, kahi helu International Prognostic Index (NCCN-IPI) i hoʻololi ʻia o 5, TP53 mutation, a me MCD subtype, e ʻike ana i ka hoʻihoʻi ʻana i loko o 6 mau mahina post-autologous transplantation.

Ma hope o ka bridging therapy, ua loaʻa i ka mea maʻi ka lāʻau steroid me ka pane maikaʻi ʻole. ʻO ka hoʻomaʻamaʻa ʻana ma hope, ua hoʻokomo ʻia nā antibodies monoclonal CD79 i hui pū ʻia me bendamustine a me ka mechlorethamine hydrochloride, ka hopena i ka hoʻohaʻahaʻa nui ʻana i nā pae LDH a me ka ʻike ʻana i ka emi ʻana o ka tumo.

Ma hope o ka hoʻomākaukau maikaʻi ʻana o ka lāʻau lapaʻau CAR-T, ua loaʻa ka mea maʻi i ka lymphocyte depletion (lymphodepletion) chemotherapy me ka FC regimen, e hoʻokō 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 ka mea maʻi i ke kuni, herpes zoster ma ka ʻāpana lumbar, a hoʻokiʻekiʻe ʻia ka pae serum lactate dehydrogenase (LDH) a hiki i 25.74ng/ml, e hōʻike ana i kahi maʻi maʻi ʻano hui like ʻole (AE). ). Ke noʻonoʻo nei i ka piʻi nui ʻana o ka infusion CAR-T ma muli o ka maʻi maʻi, hiki ke alakaʻi i nā hopena make, ua loaʻa i ka mea maʻi nā lāʻau antibiotic ākea ākea e uhi ana i nā pathogens like ʻole.

Ma hope o ka hoʻokomo ʻana o CAR-T, ua loaʻa ka mea maʻi i ke kuni kiʻekiʻe i ka lā infusion, e holomua ana i ka dyspnea, hemoptysis, a me ka hoʻonui ʻana i nā hōʻailona pulmonary i ka lā ʻekolu. ʻO ka angiography pulmonary venous CT i ka lā ʻelima i hōʻike ʻia i nā opacities honua-aniani a me nā loli interstitial, e hōʻoia ana i ka hemorrhage pulmonary. ʻOiai ka pale mua ʻana i nā steroids ma muli o ka hoʻopau ʻana i ka CAR-T, a me ke kākoʻo ʻana i ka mālama ʻana i ka hoʻokele anti-infection, ua hōʻike ʻia ke kūlana o ka mea maʻi.

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

Ma ka lā ʻewalu, ua hōʻike ʻo CAR-T therapy i ka maikaʻi kupaianaha. I loko o hoʻokahi mahina wale nō o ka mālama ʻana iā CAR-T, ua loaʻa i ka mea maʻi ke kala piha (CR). ʻO nā hoʻokolohua ma hope a hiki i Iulai 2023 ua hōʻoia i ka noho ʻana o ka mea maʻi ma CR, e hōʻike ana i kahi pane hohonu i ka CAR-T therapy a me ka hiki ke ho'ōla.