Ciwon sankarar lymphoblastic mai tsanani (B-ALL)-02

Halayen Shari'a:

- 19 ga Mayu, 2019: An gano cutar sankarar jini mai tsanani ta B-cell lymphoblastic leukemia (B-ALL)

- Yana da ciwon kai da yawa da kuma ciwon lymphadenopathy

- Tsarin jini: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, lymphocytes marasa daidaituwa 4%

- Tsarin ƙashi: kashi 80.2% na ƙwayoyin lymphoblasts marasa girma (busassun ƙwayoyin cuta)

- Tsarin Immunophenotyping: Kashi 74.19% na ƙwayoyin halitta ƙwayoyin B ne masu haɗari waɗanda ke bayyana CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, waɗanda ke bayyana cIgM a wani ɓangare. Ganewar asali: B-DUKA (Matsayin farko na B)

- Haɗaɗɗen kwayar halitta: MLL-ENL mai kyau, allon Philadelphia mai kama da chromosome (kamar Ph-like) mara kyau

- Kwayar halittar jini: 46, XX, t(11;19)(q23;p13), del(20)(q12) [3]/46, XX [7]

- Tsarin maganin chemotherapy na VDLD da farko ya sami sassaucin rigakafi bayan wata 1, MLL-ENL quantitative PCR 0.026%

- Ci gaba da shan maganin chemotherapy kamar yadda aka tsara a cikin ka'idar yara, MLL-ENL quantitative PCR 0 bayan zagaye na 4. An ci gaba da shan maganin chemotherapy.

- Maris 2020: Sauran cututtukan garkuwar jiki na ƙashi 0.35%, MLL-ENL quantitative PCR 0.53%, wanda ke nuna yanayin komawa baya. Iyali sun ƙi dashen. Ci gaba da maganin chemotherapy na tsawon zagaye 3.

- Yuli 2020: Bargon ƙashi ya sake dawowa gaba ɗaya.

- Nuwamba 11, 2020: Ciwon chemotherapy na ciki, cutar da ta rage garkuwar jiki ta CSF kashi 66%, an gano tana da cutar sankarar jini ta tsarin jijiyoyi na tsakiya. An maimaita maganin chemotherapy na ciki sau biyu, CSF ya koma mara kyau.

- Disamba 31, 2020: An kwantar da ni a asibiti.

- Tsarin jini: WBC 3.99 x 10^9/L, HGB 66 g/L, PLT 57 x 10^9/L

- Adadin fashewar jini a gefen jiki: 69%

- Siffar ƙashi: kashi 90% na ƙwayoyin lymphoblasts marasa girma (busassun ƙwayoyin cuta)

- Tsarin Immunophenotyping: Kashi 84.07% na ƙwayoyin halitta suna bayyana CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, suna bayyana CD24, CD15dim kaɗan, wanda ke nuna mummunan ƙwayoyin lymphoblasts B marasa girma.

- Tsarin halittar haɗuwa: Tsarin halittar haɗuwar MLL-ENL mai kyau, PCR mai ƙima 44.419%

- Sauyin Halitta: Sauyin KMT2D mai kyau (asalin germline)

- Karyotype na chromosome: 46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3), ƙara(14)(q34), -17, +mar [7]/46, idem, t(3;16)(p21;p13.3) [1]/46, XX [13]

- PET-CT: Yaɗuwar metabolism a cikin dukkan kwarangwal da ƙashi, babban zargin cutar sankarar bargo; splenomegaly tare da ƙaruwar metabolism, wataƙila ya shafi cutar sankarar bargo.

- An yi hudawar lumbar da kuma chemotherapy na intrathecal sau ɗaya, babu wani rashin lafiya da aka samu a gwaje-gwajen da suka shafi CSF.

Magani:

- Makonni biyu na maganin VLP, zubar jini a gefen jiki ya ragu da kashi 5% a ranar 18 ga Janairu.

- Janairu 25: Jinin da ke kewaye ya fashe da kashi 91%, an yi masa maganin CTX, Ara-C, da kuma maganin chemotherapy na 6-MP.

- 3 ga Fabrairu: Jini a gefen hanya ya fashe da kashi 22%.

- Fabrairu 4: Tarin jinin da ke kewaye da mutum 50ml don haɓaka ƙwayoyin CD19-CART.

- MTX 1g, FC chemotherapy (Mai fama da mura 15mg kowace rana x kwana 3, CTX 0.12g kowace rana x kwana 3).

- 13 ga Fabrairu (kafin a yi amfani da shi): Tsarin bargo na ƙashi yana nuna fashewar kashi 87.5%, kuma kwararar cytometry yana nuna fashewar cutar 79.4% mai tsanani.

- Nazarin adadi na haɗin kwayar halitta ta MLL-ENL: 42.639%.

- 14 ga Fabrairu: Jiƙa ƙwayoyin CART a cikin adadin 5 x 10^5/kg.

- illolin da suka shafi CAR-T: CRS na aji 1 (zazzabi), babu guba a cikin jijiyoyi.

- Rana ta 20 bayan allurar: Smear jini yana nuna yaduwar ciwon daji, rabon ƙwayoyin CART 0.07%.

- Maganin ƙwayoyin CART marasa tasiri.

- Maris 8, 2021: Tsarin jini: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.

- Fashewar jini a gefen jiki: 83%. An tattara jinin da ke kewaye da kansa 60ml don ƙwayoyin CD19/CD22 guda biyu na CART.

- Ana maganin cytarabine da dexamethasone don rage nauyin ciwon daji.

- Maris 18: Maganin chemotherapy na FC (Mai fama da mura 15mg kowace rana x kwana 3, CTX 0.12g kowace rana x kwana 3).

- Maris 22 (kafin a yi amfani da shi): Tsarin jini: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. Tsarin jinin gefe: 6% fashewa.

- Tsarin ƙashi: fashewar kashi 91%. Sauran ƙwayoyin halitta a cikin ƙashi: 88.61% suna bayyana CD38, CD19, cCD79a, CD81, CD22, suna nuna mummunan ƙwayoyin lymphoblasts B marasa girma.

- Nazarin adadi na haɗin kwayar halitta ta MLL-ENL: 62.894%.

- Binciken karyotype na chromosome: 46, XX, del(1)(p36.1), del(11)(q13), t(11;19)(q23;p13.3), ƙara(14)(q34), -17, +mar [2]/46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3), ƙara(14)(q34).

- Maris 23: Jiƙa ƙwayoyin CART a cikin allurai 3 x 10^5/kg.

- Maris 26 zuwa gaba: Zazzabi mai tsanani da kuma kumburin jiki wanda daga baya ya fara.

- Maris 29: Tsarin jinin da ke kewaye: fashewar 92%; ƙaruwar transaminases da bilirubin.

- 2 ga Afrilu: Fara farfadiya, ana yi wa magani da diazepam.

- Afrilu 2 (Rana ta 10): An fara shan maganin methylprednisolone na tsawon kwana 3.

- Haɗarin CRS: Aji na 3, CRES: Aji na 3.

- Afrilu 8 (Rana ta 16): Kimantawar ƙashi yana nuna cikakkiyar sassaucin siffa, rashin daidaituwar kwararar ƙwayoyin cuta ga fashewar ƙwayoyin cuta; nazarin ƙididdigar ƙwayoyin cuta na MLL-ENL: 0.