Indwara ya Lymphoblastique ikaze (T-BYOSE) -01

Ibiranga ivuriro:

- Gusuzuma: T-selile lymphoblastique lymphoma / leukemia

- Werurwe 2020: Yashyikirijwe inkorora ya paroxysmal hamwe na misa ya mediastinal, yemeje lymphoma T-selile lymphoma na biopsy ya mediastinal mass puncture.

- Yakiriye inzinguzingo 8 za chimiotherapie hamwe ninshuro zirenga 20 za radiotherapi, bigatuma igabanuka ryinshi rya mediastinal.

- Ku ya 16 Mutarama 2021: Yateje ububabare mu gihimba cyo hepfo.

- Gahunda y'amaraso: WBC 122.29 x 10 ^ 9 / L, HGB 91 g / L, PLT 51 x 10 ^ 9 / L

- Amagufwa ya marphologiya: 95.5% lymphoblasts yibanze.

- Amagufa yo mu magufa ya cytometrie: 91,77% by'utugingo twari lymphoblasts T-selile idakuze.

- Urukurikirane rw'irondakoko: Guhinduka muri NOTCH1, IL7R, ASXL2 byagaragaye.

- Yakiriye gahunda ya Hyper-CVAD / B, gahunda ya ESHAP nyuma, byombi ntacyo bikora hamwe numuriro uhoraho.

- Ku ya 18 Gashyantare 2021: Yinjiye mu bitaro byacu.

- Yerekanwe numuriro, igituza CT yerekanye umusonga.

- Gahunda y'amaraso: WBC 2.89 x 10 ^ 9 / L, HGB 57.7 g / L, PLT 14.9 x 10 ^ 9 / L

- Amaraso ya periferique adakuze: 90%

- Amagufa ya marrow morphologie: Hypercellular (IV grade), 85% lymphoblasts yibanze.

- Immunophenotyping: 87.27% ya selile yari malimant primitive T-selile lymphoblasts.

- Isesengura rya Chromosomal: 46, XX [24]; bitatu byiyongera karyotypes idasanzwe.

- Guhindura ingirabuzima fatizo:

1. IL7R T244_I245insARCPL ihinduka ryiza

2. ICYITONDERWA1 E1583_Q1584dup ihinduka ryiza

3. ASXL2 Q602R ihinduka ryiza

- Leukemia fusion gene yerekana: Ibibi

- PET / CT ibisubizo: Nta hypermetabolike yibibyimba yibanze muri skeleton yose hamwe no mumagufwa.

Umuti:

- Yatangiye VP reimen chimiotherapie, birambuye kuburyo bukurikira: Vincristine (VDS) 3mg inshuro imwe, Dexamethasone (Dex) 7mg buri masaha 12 muminsi 9, hamwe no kuvura indwara zanduza.

- 1 Werurwe: Utugingo ngengabuzima tutarakura twagabanutse kugera kuri 7%.

- 4 Werurwe: Yakusanyije lymphocytes autologique ya CD7-CAR T.

- 8 Werurwe: Gutangira gahunda ya VLP ihujwe no kuvura Sida benzamine.

- 14 Werurwe: Yakiriye imiti ya chimiotherapie ya FC (Fludarabine 0.35g muminsi 3, Cyclophosphamide 45mg muminsi 3).

- Ku ya 17 Werurwe (pre-selile infusion):

- Amagufwa ya marrow asigaye immunophenotyping: 15.14% selile zigaragaza CD7 nziza, CD3 dim, cytoplasmeque CD3, reseptor ya T yagabanije delta (TCRrd), kwerekana igice cya CD99, byerekana selile mbi ya primite T.

- 19 Werurwe: Yashizwemo CD7-CAR T selile (1 x 10 ^ 6 / kg).

- Ingaruka zijyanye na CAR-T: Icyiciro cya 1 CRS (umuriro), nta neurotoxicity.

- Ku ya 6 Mata (Umunsi wa 17): Amagufa ya marrow morphologie yerekanaga ko yakuweho, cytometrike itemba ntiyabonye ingirabuzimafatizo mbi.