Kala firdhiso lymfoma B-cell weyn (DBCL), nooc-hoosaadka xarunta non-germinal, oo ku lug leh daloolka sanka iyo sinuses-02

Bishii Maarso 2021, nin bukaan ah oo 52 jir ah oo ka yimid Waqooyi Bari Shiinaha ayaa soo bandhigay sanka sanka oo la helay intii lagu jiray baaritaan caadi ah. Waxa uu la kulmay calaamadaha sanka oo ciriiri ah, madax-xanuun, aragga oo xumaada, dhidid habeenkii ah, oo aan lahayn qandho ama miisaan dhimis.

Baadhitaanadii hore waxay daaha ka qaadeen cufnaanta unug jilicsan oo ballaaran oo ku lug leh sanka saxda ah ee sanka iyo sinuska, saameynaya qaababka muhiimka ah sida orbit-ka, saldhigga hore ee dhafoorka, sinuska sphenoid, iyo sinus ethmoid bidix ee MRI. Baadhitaanka cudurrada cilmu-nafsiga ee sinuska maxillary ee saxda ah ayaa soo jeediyay in la faafiyo lymphoma-ka weyn ee B-cell (DBCL), nooc-hoosaadka dhexe ee aan-germinal ahayn.

Immunohistochemistry (IHC) waxay muujisay soo galitaanka sare ee Ki-67 (90%+), CD20 (+), c-Myc (> 80%+), Bcl-2 (> 90%), Bcl-6 (+) , CD10 (-), Mum1 (+), CD79a (+), CD30 (-), iyo CyclinD1 (-), oo aan la ogaan karin Epstein-Barr fayraska RNA (EBER) yar.

Fluorescence in situ hybridization (FISH) ayaa la ogaaday Bcl-6 iyo c-myc translocations, laakiin ma jiro beddelaad hiddo-wade Bcl-2. Isku xigxiga jiilka soo socda (NGS) ayaa xaqiijiyay isbeddelada MYD88, CD79B, IGH-MYC, BAP1, iyo TP53 hiddo-wadaha, taas oo muujinaysa heerka sare ee B-cell lymphoma oo leh MYC iyo BCL2 iyo/ama BCL6.

Positron emission tomografi-computed tomografi (PET-CT) ayaa muujinaya cufnaanta unug jilicsan oo aan joogto ahayn oo ku jira daloolka sanka ee saxda ah iyo sinuska sare, qiyaastii 6.3x3.8cm cabbir ahaan, oo leh xuduudo aan kala caddayn. Nabarku waxa uu kor u fidiyay sinus-ka saxda ah ee ethmoid sinus, oo dibadda ah ilaa gidaarka dhexe ee wareegga wareegga iyo intraorbital, iyo xagga dambe ee sinuska sphenoid iyo saldhigga madaxa. Nabarku wuxuu muujiyay korodhka fluorodeoxyglucose (FDG) kor u qaadista SUVmax ee 20. Dhumucda xuubka ayaa lagu xusay ethmoid bidix iyo sinus sare, oo leh dheef-shiid kiimikaad FDG ah.

Bukaanku wuxuu hore u maray R2-CHOP, R-ESHAP, BEAM + ASCT, iyo shucaaca maxalliga ah, iyadoo la arkay horumarka cudurka. Sababtoo ah iska caabbinta kiimoterabiga iyo ka-qaybgalka badan ee xubnaha badan (oo ay ku jiraan sambabada, beerka, beeryarada, iyo lafaha), bukaanka waxaa laga helay DLBCL aasaasiga ah. Cudurku wuxuu si degdeg ah ugu socday soo galitaanka sare, heerarka LDH oo sarreeya, tusmada saadaasha Caalamiga ah ee wax laga beddelay (NCCN-IPI) dhibcaha 5, beddelka TP53, iyo nooc-hoosaadka MCD, oo la kulma soo noqoshada gudaha 6 bilood ka dib tallaalka autologous.

Daawaynta isku xidhka ka dib, bukaanku wuxuu si kooban u helay daawaynta isteeroodh oo leh jawaab xumo. Daawaynta dambe waxaa ka mid ahaa CD79 monoclonal antibodies oo ay weheliso bendamustine iyo mechlorethamine hydrochloride, taasoo keentay hoos u dhac weyn oo ku yimid heerarka LDH iyo hoos u dhac muuqda oo burooyinka.

Ka dib markii si guul leh loo diyaariyo daaweynta CAR-T, bukaanku wuxuu maray kiimoterabi hoos u dhaca (lymphodepletion) kiimoterabiga oo leh nidaamka FC, gaaritaanka nadiifinta lymphocyte ee loogu talagalay iyo leukopenia daran ee xigtay. Si kastaba ha ahaatee, saddex maalmood ka hor faleebo CAR-T, bukaanku wuxuu yeeshay qandho, herpes zoster ee gobolka lumbar, iyo heerarka serum lactate dehydrogenase (LDH) oo sarreeya ilaa 25.74ng / ml, taas oo muujinaysa dhacdo khatar ah oo isku dhafan oo nooc ah oo firfircoon (AE). ). Iyadoo la tixgelinayo khatarta sii kordheysa ee faleebo CAR-T oo ay ugu wacan tahay infekshanka firfircoon, oo laga yaabo inuu keeno natiijooyin dhimasho ah, bukaanku wuxuu helay antibiyootiko ballaaran oo daboolaya cudurro kala duwan.

Ka dib faleebo CAR-T ah, bukaanku wuxuu yeeshay qandho sare maalinta faleebada, oo u gudbaya dyspnea, hemoptysis, iyo calaamadaha sambabada oo sii xumaanaya maalinta saddexaad. CT angiography ee xididada sambabada maalintii shanaad ayaa daaha ka qaaday madhnaanshiyaha muraayadaha dhulka oo kala firdhiyey iyo isbeddelada dhexmara, taasoo xaqiijinaysa dhiig-baxa sambabada. Inkasta oo laga fogaado bilowga hore ee steroids sababtoo ah xakamaynta CAR-T ee suurtogalka ah, iyo daaweynta taageerada ee diiradda saaraya maareynta caabuqa, xaaladda bukaanku waxay muujisay horumar xaddidan.

Maalintii toddobaad, balaadhinta nambarka nuqulka hiddasidaha CAR ee muhiimka ah ayaa laga helay dhiigga ku wareegsan, taasoo keentay hagaajin daawaynta oo leh methylprednisolone-yar (40mg-80mg). Shan maalmood ka dib, sambabada laba geesoodka ah ayaa hoos u dhacay, calaamadaha hemoptysis ayaa si gaar ah loo xakameeyay.

Maalintii siddeedaad, daawaynta CAR-T waxay muujisay waxtarka cajiibka ah. Hal bil gudaheed ee daaweynta CAR-T, bukaanku wuxuu helay cafis dhammaystiran (CR). Baaritaanadii xigay ilaa Luulyo 2023 ayaa xaqiijiyay in bukaanku uu ku sii jiro CR, taasoo muujineysa jawaab qoto dheer oo ku saabsan daaweynta CAR-T iyo suurtagalnimada daawaynta.