Te lymphoma pūtau-B nui horapa (DLBCL), te momo pokapū kore-germinal, e pā ana ki te kōhao ihu me ngā sinuses-02

I te marama o Poutū-te-rangi 2021, i tae mai tētahi tūroro tāne 52-tau te pakeke nō te raki-mā-rāwhiti o Haina me te ihu pupuhi i kitea i te wā o te tirotiro ā-tau. I pāngia ia e ngā tohu o te ihu pupuhi, te ānini, te tirohanga pōuri, me te werawera i te pō, engari kāore he kirika, he whakaheke taumaha rānei.

I roto i ngā whakamātautau tuatahi, i kitea he nui te puranga kiko ngohengohe e pā ana ki te kōhao ihu matau me ngā sinus, ā, ka pā ki ngā hanganga nui pērā i te porowhita, te turanga angaanga o mua, te sinus sphenoid, me te sinus ethmoid maui i runga i te MRI. I whakaatuhia e te whakamātautau pathological o te sinus maxillary matau he lymphoma pūtau-B nui horapa (DLBCL), he momo pokapū kore-germinal.

I whakaatuhia e te Immunohistochemistry (IHC) te kaha whakaeke nui me te whakaaturanga takirua o Ki-67 (90%+), CD20 (+), c-Myc (>80%+), Bcl-2 (>90%), Bcl-6 (+), CD10 (-), Mum1 (+), CD79a (+), CD30 (-), me CyclinD1 (-), me te kore he RNA iti (EBER) e whakakōnaehia ana e te huaketo Epstein-Barr.

I kitea e te fluorescence in situ hybridization (FISH) ngā whakawhiti ira Bcl-6 me c-myc, engari kāore he whakawhiti ira Bcl-2. I whakaūtia e te raupapatanga whakatupuranga e whai ake nei (NGS) ngā whakarerekētanga ira i roto i ngā ira MYD88, CD79B, IGH-MYC, BAP1, me TP53, e tohu ana i te lymphoma pūtau-B taumata teitei me ngā whakawhiti ira MYC me BCL2 me/ranei BCL6.

I whakaatuhia e te tomography tukunga positron-tomography rorohiko (PET-CT) ngā puranga kiko ngohengohe koretake i roto i te kōhao ihu matau me te sinus o runga, tata ki te 6.3x3.8cm te rahi, me ngā taha kore e mārama. I toro atu te whara ki runga ki roto i te sinus ethmoid matau, ki waho ki te pakitara waenga o te orbit me te rohe intraorbital, ā, ki muri ki te sinus sphenoid me te turanga angaanga. I whakaatuhia e te whara te pikinga o te tango fluorodeoxyglucose (FDG) me te SUVmax o te 20. I kitea te matotoru o te mucosal i roto i te sinus ethmoid maui me te sinus o runga, me te metabolism FDG noa.

I mua, kua whiwhi te tūroro i te R2-CHOP, R-ESHAP, BEAM+ASCT, me te radiotherapy ā-rohe, ā, i kitea te whanaketanga o te mate. Nā te ātete ki te chemotherapy me te nui o te pānga o ngā okana maha (tae atu ki ngā pukahukahu, te ate, te whēkau, me ngā wheua), i kitea te mate o te tūroro me te DLBCL ātete tuatahi. I tere te whanaketanga o te mate me te kaha whakaeke nui, ngā taumata LDH teitei, te kaute NCCN-IPI kua whakarerekētia (NCCN-IPI) o te 5, te whakarerekētanga TP53, me te momo MCD, ā, ka hoki anō ki te ora i roto i ngā marama e 6 i muri i te whakawhitinga uri.

Whai muri i te rongoā honohono, i whiwhi te tūroro i te rongoā steroid mō te wā poto, engari kāore i pai te urupare. I muri mai, i whakaurua ngā paturopi monoclonal CD79 ki te bendamustine me te mechlorethamine hydrochloride, ā, i hua ake ai he whakahekenga nui o ngā taumata LDH me te whakahekenga kitea o te pukupuku.

I muri i te angitu o te whakarite i te rongoā CAR-T, i tukuna te rongoā chemotherapy mō te whakaiti i ngā pūtau toto (lymphodepletion) ki te tūroro me te rongoā FC, ā, i tutuki ai te whakakorenga o ngā pūtau toto i whakaritea, ā, i muri mai ka nui te leukopenia. Heoi, e toru ngā rā i mua i te werohanga o te CAR-T, ka pāngia te tūroro e te kirika, te herpes zoster i te rohe hope, me te pikinga o ngā taumata lactate dehydrogenase (LDH) serum ki te 25.74ng/ml, e tohu ana i tētahi mate urutā momo whakauru pea (AE). I te whakaaro ki te pikinga ake o te mōrearea o te werohanga CAR-T nā te mate urutā, ka arahi pea ki ngā hua mate, i whiwhi te tūroro i ngā paturopi whānui-ahu e kapi ana i ngā momo pathogens.

Whai muri i te werohanga CAR-T, ka pāngia te tūroro e te kirika nui i te rā werohanga, ka haere tonu ki te mate manawa, te hemoptysis, ā, ka kino haere ngā tohu o te pūkahukahu i te rā tuatoru. I whakaatuhia e te angiography CT venous pūkahukahu i te rā tuarima ngā opacities ground-glass me ngā huringa interstitial, e whakaū ana i te totohu pūkahukahu. Ahakoa te karo tuatahi i ngā steroids nā te mea ka taea te pehi i te CAR-T, me te maimoatanga tautoko i arotahi ki te whakahaere i te mate, he iti noa te whakapainga o te āhua o te tūroro.

I te whitu o ngā rā, i kitea te pikinga nui o te maha o ngā tārua ira CAR i roto i te toto ā-taha, ā, i akiakihia kia whakatikatikaina te maimoatanga me te horopeta iti o te methylprednisolone (40mg-80mg). E rima ngā rā i muri mai, i heke te tangi o ngā pūkahukahu e rua, ā, i tino whakahaeretia ngā tohu hemoptysis.

I te waru o ngā rā, i kitea he tino whai huatanga o te rongoā CAR-T. I roto noa i te kotahi marama o te rongoā CAR-T, i tutuki i te tūroro te murunga katoa (CR). Nā ngā whakamātautau i muri mai tae noa ki te Hūrae 2023 i whakaū kei te noho tonu te tūroro i roto i te CR, e tohu ana i te urupare hohonu ki te rongoā CAR-T me te kaha ki te ora.