Indwara ya Lymphoblastique ikaze (T-BYOSE) -02

Ibiranga ivuriro:

- Gusuzuma: Indwara ya T-selile lymphoblastique leukemia

- Gutangira: Mu mpera za Werurwe 2018

- Ibimenyetso byambere: Kwiyongera kwinshi kwa lymph node kwaguka mumubiri

- Gahunda yambere yamaraso: WBC: 39.46 * 10 ^ 9 / L, Hb: 129g / L, PLT: 77 * 10 ^ 9 / L

- Amagufwa yo mu magufa: guturika 92%

-Fata cytometrie: 95.3% selile zidasanzwe zerekana

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

- Fusion genes: Ibibi

- Guhinduranya gene: NOTCH1 gene mutation yagaragaye

- Isesengura rya Chromosome: Karyotype isanzwe

Amateka yo kuvura:

- Ku ya 3 Mata 2018: Ubuvuzi bwa Induction hamwe na gahunda ya VDCP

- Ku ya 18 Mata 2018: Guturika kw'amagufwa bigizwe na 96%

- Ku ya 20 Mata 2018: Yagezweho gukosorwa nyuma ya CAG

- Ku ya 18 Gicurasi 2018: Ubuvuzi bwo guhuza hamwe na gahunda ya CMG + VP

- Ku ya 22 Kamena 2018: Guturika kw'amagufwa yiyongereye kugera kuri 40%, kongera kurwara leukemia

- Ku ya 25 Nyakanga 2018: Gahunda ya CLAM (clarithromycin + cyclophosphamide + amikacin)

- Hematopoietic stem selile transplantation kuva murumuna wa HLA uhuye ukoresheje FLU + BU ikonjesha ku ya 14 Kanama

- Gukurikirana nyuma yo kwimurwa: Gukuraho amagufwa ya morfologiya ukwezi 1, amezi 3, amezi 6, amezi 9, n'amezi 11

- Amagufwa ya marrow morphologie yerekanaga ko yakiriwe nyuma y'amezi 16 nyuma yo guterwa, hamwe na cytometrike yerekana ko 0,02% ya lymphocytes idakuze;

- Ku ya 13 Ugushyingo 2020: Chimerism ya periferique ituruka ku baterankunga yari 97.9%

- Amaraso ya periferique primitive selile: 20%

- Ku ya 18 Ukuboza 2020: Amagufwa yo mu magufa: guturika 60,6%

- Flow cytometrie: 30,85% malignant idakuze T lymphocytes

- Isesengura rya Chromosome: 46, XY (20)

- Yakiriye DA regimen chimiotherapie ku ya 19 Mutarama 2021

- Amagufwa yo mu magufa ku ya 19 Mutarama 2021: hyperplasia yo mu cyiciro cya III, ibisasu 16%

- Isesengura rya Chromosome karyotype: 46, XY (20)

- Flow cytometrie: 7.27% by'utugingo ngengabuzima (mu ngirabuzimafatizo za kirimbuzi) bagaragaje CD99bri, CD13, CD38, cbcl-2, cCD3, HLA-ABC bri, CD7bri, kandi bagaragaza CD5dim igice, byerekana lymphocytes T idakuze;

- Leukemia fusion gene yerekana: Ibibi

- Isesengura ryimiterere yamaraso (ubwoko 86):

1. PHF6 K299Efs * 13 ihinduka ryiza

2. RUNX1 S322 * ihinduka ryiza

3. FBXW7 E471G ihinduka ryiza

4. JAK3 M511I ihinduka ryiza

5. ICYITONDERWA1 Q2393 * ihinduka ryiza

Umuti:

- 22 Mutarama: Gukusanya n'umuco bya autologique peripheral maraso lymphocytes ya CD7-CART

- Mbere yo kwinjiza CD7-CART, umurwayi yakiriye VLP (vincristine, l-asparaginase, prednisone) wongeyeho chimiotherapie ya bortezomib.

- 3 Gashyantare: FC regimen chimiotherapie (ibicurane 50mg muminsi 3 + CTX 0.45g muminsi 3)

- 5 Gashyantare (pre-infusion): Amagufa ya marrow morphologie yerekanaga 23%.

- Flow cytometrie yerekanye selile 4.05% zerekana CD99bri, CD5dim, CD7bri, TDT, cCD3, byerekana lymphocytes T idakuze.

- Isesengura rya Chromosome: 46, XY (20)

- Isesengura rya Chimerism (nyuma ya HSCT): Ingirabuzimafatizo zikomoka ku baterankunga zingana na 52.19%.

- 7 Gashyantare: Kwinjiza ingirabuzimafatizo za CD7-CART ku kigero cya 5 * 10 ^ 5 / kg.

- 15 Gashyantare: Amaraso ya periferique adakuze yagabanutse kugera kuri 2%.

- 19 Gashyantare (Umunsi wa 12 nyuma yo gushiramo): Umurwayi yagize umuriro, wamaraga iminsi 5 mbere yo kugenzura ubushyuhe.

- Tariki ya 2 Werurwe: Isuzuma ry'amagufwa ryerekanaga ko ryuzuye ryuzuye, hamwe na cytometrike itemba itagaragaza ingirabuzimafatizo zidakuze.