Chirwere cheAcute Lymphoblastic Leukemia (T-ALL)-02

Zvimiro zveKliniki:

- Kuongororwa: Acute T-cell lymphoblastic leukemia

- Kutanga: Kupera kwaKurume 2018

- Zviratidzo zvekutanga: Kuwedzera kwema lymph node mumuviri wese

- Kuongororwa kwekutanga kweropa: WBC: 39.46*10^9/L, Hb: 129g/L, PLT: 77*10^9/L

- Maumbirwo emapfupa: 92% kuputika

-Flow cytometry: 95.3% masero asina kujairika anoratidza

TdT+CD99+CyCD3+CD7stCd5DdimCD4-CD8-mCD3-CD45dim

- Majini eFusion: Hasi

- Kuchinja kwemajini: Kuchinja kwemajini kweNOTCH1 kwakaonekwa

- Kuongorora ma chromosome: Karyotype yakajairika

Nhoroondo yeKurapwa:

- Kubvumbi 3, 2018: Kurapa kwemushonga weVDCP

- Kubvumbi 18, 2018: Kuputika kwemapfupa kwaive ne96%

- Kubvumbi 20, 2018: Ndakawana zororo mushure mekushandisa CAG

- Chivabvu 18, 2018: Kurapa kwakabatana neCMG+VP regimen

- Chikumi 22, 2018: Kuputika kwemapfupa kwakawedzera kusvika 40%, kudzoka kwechirwere cheleukemia

- Chikunguru 25, 2018: Mushonga weCLAM (clarithromycin+cyclophosphamide+amikacin)

- Kuisirwa masero eropa kubva kuHLA-matched sibling uchishandisa FLU+BU conditioning musi wa14 Nyamavhuvhu

- Kutarisa mushure mekuisirwa: Kubviswa kwechimiro chemubofu mumongo pamwedzi 1, mwedzi 3, mwedzi 6, mwedzi 9, uye mwedzi 11

- Maumbirwo emapfupa emubofu akaratidza kurerukirwa mushure memwedzi gumi nemitanhatu mushure mekuisirwa mwana, uye flow cytometry yakaratidza 0.02% yemasero emubofu asina kunaka (malignant immature lymphocytes).

- Mbudzi 13, 2020: Kushandiswa kweropa rakawanda kubva kune vanopa ropa kwaive 97.9%

- Masero ekutanga eropa ekunze: 20%

- Zvita 18, 2020: Maumbirwo emapfupa: 60.6% kuputika

- Flow cytometry: 30.85% malignant immature T lymphocytes

- Kuongorora ma chromosome: 46, XY (20)

- Akapihwa chemotherapy yeDA musi wa19 Ndira, 2021

- Maumbirwo emapfupa musi wa19 Ndira, 2021: Kukura kweGrade III hyperplasia, 16% kuputika

- Kuongororwa kwekaryotype yechromosome: 46, XY (20)

- Flow cytometry: 7.27% yemasero (pakati pemasero enyukireya) anoratidzwa CD99bri, CD13, CD38, cbcl-2, cCD3, HLA-ABC bri, CD7bri, uye CD5dim akabudiswa zvishoma, zvichiratidza malignant immature T lymphocytes

- Kuongororwa kwemajini eLeukemia fusion: Negative

- Kuongorora kushanduka kwegomarara reropa (mhando 86):

1. PHF6 K299Efs*13 mutation positive

2. RUNX1 S322* shanduko yakanaka

3. FBXW7 E471G mutation ine positive

4. JAK3 M511I mutation ine hutachiona

5. NOTCH1 Q2393* shanduko yakanaka

Kurapa:

- Ndira 22: Kuunganidzwa uye kuumbwa kwemasero eropa ega ega eCD7-CART

- Murwere asati apihwa CD7-CART, akapihwa VLP (vincristine, l-asparaginase, prednisone) pamwe chete ne bortezomib chemotherapy.

- Kukadzi 3: Kurapwa kweFC chemotherapy (Flu 50mg kwemazuva matatu + CTX 0.45g kwemazuva matatu)

- Kukadzi 5 (kusati kwaiswa mushonga): Maumbirwo emapfupa akaratidza kuputika kwe23%.

- Flow cytometry yakaratidza 4.05% yemasero ane CD99bri, CD5dim, CD7bri, TDT, cCD3, zvichiratidza kuti T lymphocytes dzisina kukura dzakaipa.

- Kuongorora ma chromosome: 46, XY (20)

- Kuongororwa kweChimerism (post-HSCT): Masero anobva kune vanopa aisvika 52.19%.

- Kukadzi 7: Kuiswa kwemasero eCD7-CART e autologous pachiyero che5*10^5/kg.

- Kukadzi 15: Masero eropa asina kukura akaderera kusvika pa2%.

- Kukadzi 19 (Zuva rechi12 mushure mekunwa mushonga): Murwere akatanga fivha, iyo yakagara kwemazuva mashanu isati yasvika pakudzora tembiricha.

- Kurume 2: Kuongororwa kwemapfupa kwakaratidza kurerukirwa kwakakwana kwechimiro, nepo flow cytometry isingaone masero asina kukura ane kenza.