Chirwere cheAcute Lymphoblastic Leukemia (B-ALL)-02

Hunhu hweNyaya:

- Chivabvu 19, 2019: Akaonekwa aine chirwere cheB-cell lymphoblastic leukemia (B-ALL)

- Inoratidzwa nemapoka akawanda eganda remusoro uye lymphadenopathy

- Ropa rinoitwa nguva dzose: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, lymphocytes dzisina kujairika 4%

- Maumbirwo emubofu: 80.2% lymphoblasts dzisina kukura (blasts)

- Immunophenotyping: 74.19% yemasero masero eB-lineage precursor ane malignant ane CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, anowedzera zvishoma cIgM. Kuongorora: B-ALL (Pre-B stage)

- Jini reFusion: MLL-ENL yakanaka, Philadelphia yakafanana nekromosomu (Ph-yakafanana) isina kunaka pachiratidziro

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

- VDLD regimen chemotherapy yakatanga yawana relieve ye immunological mushure memwedzi mumwe chete, MLL-ENL quantitative PCR 0.026%

- Kuramba uchipiwa chemotherapy zvichienderana neprotocol yevana, MLL-ENL quantitative PCR 0 mushure mekuongororwa kwechikamu chechina. Kuenderera mberi nekupihwa chemotherapy.

- Kurume 2020: Chirwere che immunological residual disease che bone marrow 0.35%, MLL-ENL quantitative PCR 0.53%, zvichiratidza kuti chirwere ichi chatanga kudzokera shure. Mhuri yakaramba kuisirwa mushonga. Yakaramba ichipiwa chemotherapy kwemavhiki matatu.

- Chikunguru 2020: Bone mongo yakadzoka zvachose.

- Mbudzi 11, 2020: Kurapwa kwemishonga yemukati memushonga, CSF immunological residual disease 66%, yakawanikwa ine central nervous system leukemia. Kurapwa kwemishonga yemukati memushonga kwakadzokororwa kaviri, CSF ikaonekwa isina.

- Zvita 31, 2020: Ndakapinda muchipatara chedu.

- Ropa rinoitwa nguva dzose: WBC 3.99 x 10^9/L, HGB 66 g/L, PLT 57 x 10^9/L

- Huwandu hweropa rinoputika muropa: 69%

- Maumbirwo emubofu: 90% lymphoblasts dzisina kukura (blasts)

- Immunophenotyping: 84.07% yemasero ane CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, ane CD24, CD15dim zvishoma, zvichiratidza kuti maB lymphoblasts asina kukura zvakanaka.

- Jini reFusion: MLL-ENL fusion gene yakanaka, PCR yakawanda 44.419%

- Kuchinja kwemajini: Kuchinja kweKMT2D kwakanaka (kwakabva germline)

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

- PET-CT: Kuwedzera kwemetabolism mumapfupa ese nemapfupa, fungidziro yakakura yekuti leukemia ichadzokazve; splenomegaly ine kuwedzera kwemetabolism, pamwe ichisanganisira leukemia.

- Ndakaita lumbar puncture uye intrathecal chemotherapy kamwe chete, hapana chakaipa chakawanikwa mukuongororwa kwakabatana neCSF.

Kurapa:

- Mavhiki maviri eVLP chemotherapy, ropa rinobuda muropa rinokwira ne5% musi wa18 Ndira.

- Ndira 25: Ropa rekunze rakaputika ne91%, rakarapwa neCTX, Ara-C, 6-MP chemotherapy.

- Kukadzi 3: Kuputika kweropa rekunze kwenzvimbo dze ...

- Kukadzi 4: Kuunganidzwa kwe50ml yeropa remuviri rinobuda muropa kuti rikwanise kuumbwa neCD19-CART cell.

- MTX 1g, FC chemotherapy (Flu 15mg zuva nezuva x mazuva matatu, CTX 0.12g zuva nezuva x mazuva matatu).

- Kukadzi 13 (kusati kwaiswa mushonga): Maumbirwo emapfupa anoratidza 87.5% yekuputika, flow cytometry inoratidza 79.4% yekuputika kunotyisa.

- Kuongororwa kwehuwandu hwemajini ekubatanidza MLL-ENL: 42.639%.

- Kukadzi 14: Kuiswa kweCART cells pachiyero che5 x 10^5/kg.

- Migumisiro yakaipa inokonzerwa neCAR-T: CRS yeGiredhi 1 (fivha), hapana chepfu muuropi.

- Zuva rechi20 mushure mekuisirwa ropa: Kuongororwa kweropa kunoratidza kuwanda kwebundu, CART cell proportion 0.07%.

- Kurapa kweCART cell kusina kushanda.

- Kurume 8, 2021: Kushandiswa kweropa: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.

- Kuputika kweropa rekunze: 83%. Ropa rekunze rakaunganidzwa 60ml kuti rikwanise kuumbwa neCD19/CD22 dual CART cell.

- Inorapwa ne cytarabine ne dexamethasone kudzora mutoro wegomarara.

- Kurume 18: FC chemotherapy (Flu 15mg zuva nezuva x mazuva matatu, CTX 0.12g zuva nezuva x mazuva matatu).

- Kurume 22 (kusati kwaiswa mushonga): Ropa rinotorwa: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. Maumbirwo eropa rekunze: 6% kuputika.

- Maumbirwo emapfupa emubofu: 91% kuputika. Zvasara mumapfupa emubofu: 88.61% masero ane CD38, CD19, cCD79a, CD81, CD22, zvichiratidza masero eB lymphoblasts asina kukura zvakanaka.

- Kuongororwa kwehuwandu hwemajini ekubatanidza MLL-ENL: 62.894%.

- Kuongororwa kwekaryotype yechromosome: 46, XX, del(1)(p36.1), del(11)(q13), t(11;19)(q23;p13.3), wedzera(14)(q34), -17, +mar [2]/46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3), wedzera(14)(q34).

- Kurume 23: Kuiswa kweCART cells pachiyero che3 x 10^5/kg.

- Kurume 26 zvichienda mberi: Fivha yakasimba inoramba iripo uye zvakazopedzisira zvaita kuzvimba mumuviri.

- Kurume 29: Maumbirwo eropa rekunze: 92% kuputika; ma transaminases akakwira uye bilirubin.

- Kubvumbi 2: Kutanga kwechirwere chepfari, chakarapwa ne diazepam.

- Kubvumbi 2 (Zuva regumi): Takatanga kurapwa nemethylprednisolone kwemazuva matatu.

- Maitiro eCRS: Giredhi 3, CRES: Giredhi 3.

- Kubvumbi 8 (Zuva re16): Kuongororwa kwemapfupa kunoratidza kusunungurwa kwakakwana kwechimiro, flow cytometry isina kunaka kune malignant blasts; MLL-ENL fusion gene quantitative analysis: 0.