Yada manyan ƙwayoyin lymphoma B-cell (DLBCL), nau'in cibiyar da ba na germinal ba, wanda ya haɗa da kogon hanci da sinuses-02

A watan Maris na shekarar 2021, wani majinyaci dan shekara 52 daga Arewa maso Gabashin kasar Sin ya gabatar da hancin hancin da aka gano a yayin da ake duba lafiyarsa na yau da kullun. Ya fuskanci alamun cunkoson hanci, ciwon kai, rashin hangen nesa, da gumin dare, ba tare da zazzaɓi ko rage nauyi ba.

Gwaje-gwaje na farko sun nuna tarin nama mai laushi mai yawa wanda ya ƙunshi rami na hanci na dama da sinuses, yana shafar sifofi masu mahimmanci irin su orbit, gindin kwanyar baya, sinus sphenoid, da kuma sinus na ethmoid na hagu akan MRI. Binciken Pathological na maxillary sinus na dama ya ba da shawarar watsa babban lymphoma B-cell (DBCL), subtype ba na tsakiya ba.

Immunohistochemistry (IHC) ya nuna babban mamayewa tare da magana biyu na Ki-67 (90%+), CD20 (+), c-Myc (> 80%+), Bcl-2 (> 90%), Bcl-6 (+) , CD10 (-), Mum1 (+), CD79a (+), CD30 (-), da CyclinD1 (-), ba tare da wani ƙaramin RNA (EBER) mai ɗauke da cutar Epstein-Barr ba.

Fluorescence in situ hybridization (FISH) ya gano fassarar Bcl-6 da c-myc, amma babu fassarar halittar Bcl-2. Matsakaicin tsararraki na gaba (NGS) ya tabbatar da maye gurbi a cikin MYD88, CD79B, IGH-MYC, BAP1, da TP53 genes, yana nuna babban darajar B-cell lymphoma tare da MYC da BCL2 da / ko BCL6 canzawa.

Positron emission tomography-computed tomography (PET-CT) yana nuna adadin nama mai laushi marasa daidaituwa a cikin kogon hanci na dama da babban sinus, kusan 6.3x3.8cm cikin girman, tare da iyakoki mara kyau. Ciwon ya kara zuwa sama zuwa sinus na ethmoid na dama, a waje zuwa bangon tsakiya na orbit da intraorbital yankin, kuma daga baya zuwa sphenoid sinus da kwanyar kwanyar. Lalacewar ta nuna ƙarar ƙwayar fluorodeoxyglucose (FDG) tare da SUVmax na 20. An lura da kauri na mucosal a cikin ethmoid na hagu da kuma sinus mafi girma, tare da al'ada FDG metabolism.

Mai haƙuri ya riga ya sha R2-CHOP, R-ESHAP, BEAM + ASCT, da kuma rediyo na gida, tare da ci gaba da cutar. Saboda juriya na chemotherapy da kuma shigar da gabobin da yawa (ciki har da huhu, hanta, saifa, da kasusuwa), an gano mai haƙuri tare da DLBCL na farko na refractory. Cutar ta ci gaba da sauri tare da ɓarna mai girma, haɓaka matakan LDH, gyare-gyaren Ƙididdigar Ƙwararrun Ƙwararru na Ƙasashen Duniya (NCCN-IPI) na 5, maye gurbi na TP53, da kuma nau'in MCD, yana fuskantar koma baya a cikin watanni 6 bayan dashen-autologous.

Bayan maganin haɗin gwiwa, mai haƙuri ya ɗan ɗan sami maganin steroid tare da amsa mara kyau. Daga baya jiyya ya haɗa da CD79 monoclonal antibodies hade da bendamustine da mechlorethamine hydrochloride, wanda ya haifar da raguwa mai yawa a cikin matakan LDH da raguwar ƙwayar ƙwayar cuta.

Bayan nasarar shirye-shiryen maganin CAR-T, mai haƙuri ya sami raguwar ƙwayar cuta ta lymphocyte (lymphodepletion) chemotherapy tare da tsarin FC, cimma nasarar da aka yi niyya na lymphocyte da kuma leukopenia mai tsanani na gaba. Duk da haka, kwanaki uku kafin jiko na CAR-T, mai haƙuri ya kamu da zazzaɓi, herpes zoster a cikin yankin lumbar, da kuma matakan haɓakar lactate dehydrogenase (LDH) har zuwa 25.74ng / ml, yana nuna yiwuwar haɗuwa da nau'in kamuwa da cuta mai haɗari (AE) ). Idan aka yi la'akari da ƙara haɗarin jiko na CAR-T saboda kamuwa da cuta mai aiki, mai yuwuwar haifar da sakamako mai ƙima, mai haƙuri ya karɓi maganin rigakafi da yawa waɗanda ke rufe ƙwayoyin cuta daban-daban.

Bayan jiko na CAR-T, mai haƙuri ya kamu da zazzaɓi mai zafi a ranar jiko, yana ci gaba zuwa dyspnea, hemoptysis, da tabarbarewar alamun huhu ta rana ta uku. CT angiography venous na huhu a rana ta biyar ya bayyana bazuwar gilasan gilasai da canje-canjen tsaka-tsaki, yana tabbatar da zubar jini na huhu. Duk da guje wa farko na steroids saboda yuwuwar CAR-T dannewa, da kuma kulawa da tallafi da aka mayar da hankali kan kula da cututtuka, yanayin mai haƙuri ya nuna ƙarancin ci gaba.

A rana ta bakwai, an gano haɓaka lambar kwafin ƙwayoyin CAR mai mahimmanci a cikin jini na gefe, yana haifar da daidaitawar jiyya tare da ƙaramin adadin methylprednisolone (40mg-80mg). Kwanaki biyar bayan haka, rales ɗin huhu na biyu ya ragu, kuma alamun cutar haemoptysis an sarrafa su sosai.

A rana ta takwas, maganin CAR-T ya nuna ingantaccen inganci. A cikin wata ɗaya kawai na maganin CAR-T, mai haƙuri ya sami cikakkiyar gafara (CR). Gwaje-gwajen da suka biyo baya har zuwa Yuli 2023 sun tabbatar da majinyacin ya kasance a cikin CR, yana nuna babban martani ga maganin CAR-T da yuwuwar warkewa.