Indwara ya Lymphoblastique ikaze (B-BYOSE) -02

Ibiranga urubanza:

- Gicurasi 19, 2019: Yasuzumwe na lymphoblastique ikaze ya B-selile (B-BYOSE)

- Yerekanwe hamwe na misa myinshi yo mumutwe hamwe na lymphadenopathie

- Gahunda y'amaraso: WBC 13.3 x 10 ^ 9 / L, HGB 94 g / L, PLT 333 x 10 ^ 9 / L, lymphocytes zidasanzwe 4%

- Amagufwa yo mu magufa: 80.2% lymphoblasts idakuze (guturika)

. Gusuzuma: B-BYOSE (Icyiciro kibanziriza B)

- Fusion gene: MLL-ENL nziza, Philadelphia chromosome isa (Ph-imeze) ecran mbi

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

- VDLD gahunda ya chimiotherapie yabanje kugera ku gukingira indwara nyuma yukwezi 1, MLL-ENL yuzuye PCR 0.026%

- Gukomeza imiti ya chimiotherapie nkuko protocole yabana yabana, MLL-ENL yuzuye PCR 0 nyuma yumuzingo wa 4. Ubundi chimiotherapie yarakomeje.

- Werurwe 2020: Indwara isigaye y’amagufwa y’amagufwa 0.35%, MLL-ENL yuzuye PCR 0.53%, byerekana inzira yo gusubira inyuma. Umuryango wanze kwimurwa. Gukomeza chimiotherapie kumuzingo 3.

- Nyakanga 2020: Amagufwa yongeye kugaruka muri rusange.

- Ku ya 11 Ugushyingo 2020: Imiti ya chimiotherapie, Indwara isigara ya CSF 66%, yasuzumwe na sisitemu yo hagati yo mu maraso. Imiti ya chimiotherapie isubirwamo kabiri, CSF yahindutse nabi.

- 31 Ukuboza 2020: Yinjiye mu bitaro byacu.

- Gahunda y'amaraso: WBC 3.99 x 10 ^ 9 / L, HGB 66 g / L, PLT 57 x 10 ^ 9 / L

- Kubara amaraso ya periferique: 69%

- Amagufwa ya marphologiya: 90% lymphoblasts idakuze (guturika)

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- Fusion gene: MLL-ENL fusion gene nziza, ingano PCR 44.419%

- Guhindura ingirabuzima fatizo: KMT2D ihinduka ryiza (inkomoko ya germine)

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

- PET-CT: Kwiyongera kwa metabolike kwiyongera mumyanya yose ya skeletale na magufa, gukeka cyane ko leukemia yongeye kubaho; splenomegaly hamwe no kwiyongera kwa metabolism, birashoboka ko harimo na leukemia.

- Gukora ibibyimba byo mu mutwe hamwe na chimiotherapie intrathecal inshuro imwe, nta bidasanzwe biboneka mu bizamini bijyanye na CSF.

Umuti:

- Ibyumweru bibiri bya chimiotherapie ya VLP, amaraso ya peripheri aturika 5% ku ya 18 Mutarama.

- 25 Mutarama: Amaraso ya periferiya yaturitse 91%, avurwa na CTX, Ara-C, chimiotherapie ya MP-6.

- 3 Gashyantare: Amaraso ya periferiya aturika 22%.

- 4 Gashyantare: Ikusanyirizo rya 50ml autologique peripheral maraso ya CD19-CART umuco w'akagari.

- MTX 1g, FC chimiotherapie (ibicurane 15mg buri munsi x iminsi 3, CTX 0.12g buri munsi x iminsi 3).

- Tariki ya 13 Gashyantare (pre-infusion): Amagufa ya marphologie yerekana 87.5% yaturika, cytometrike yerekana 79.4% iturika nabi.

- MLL-ENL fusion gene isesengura ryinshi: 42.639%.

- 14 Gashyantare: Kwinjiza selile CART ku kigero cya 5 x 10 ^ 5 / kg.

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

- Umunsi wa 20 nyuma yo gushiramo: Amaraso yamenetse yerekana ikwirakwizwa ryibibyimba, igipimo cya selile CART 0,07%.

- Ubuvuzi bwa CART butagira ingaruka.

- Ku ya 8 Werurwe 2021: Gahunda y'amaraso: WBC 38.55 x 10 ^ 9 / L, HGB 65g / L, PLT 71.60 x 10 ^ 9 / L.

- Amaraso ya periferiya aturika: 83%. Autologous peripheral blood yakusanyije 60ml kuri CD19 / CD22 ya kabiri ya CART selile.

- Bivuwe na cytarabine na dexamethasone kugirango ugabanye umutwaro wibibyimba.

- Ku ya 18 Werurwe: FC chimiotherapie (ibicurane 15mg buri munsi x iminsi 3, CTX 0,12g buri munsi x iminsi 3).

- 22 Werurwe (pre-infusion): Gahunda yamaraso: WBC 0.42 x 10 ^ 9 / L, HGB 93.70g / L, PLT 33.6 x 10 ^ 9 / L. Amaraso ya periferique morphologie: guturika 6%.

- Amagufwa yo mu magufa: guturika 91%. Ibisigarira mumagufa: 88,61% selile zigaragaza CD38, CD19, cCD79a, CD81, CD22, byerekana lymphoblasts B idakuze.

- MLL-ENL fusion gene isesengura ryinshi: 62.894%.

- Isesengura rya Chromosome karyotype: 46, XX, del (1) (p36.1), del (11) (q13), t (11; 19) (q23; p13.3), ongeraho (14) (q34), - 17, + mar [2] / 46, XX, del (1) (p36.1), del (1) (q31q42), del (11) (q13), t (11; 19) (q23; p13.3 ), ongeraho (14) (q34).

- 23 Werurwe: Kwinjiza selile CART ku kigero cya 3 x 10 ^ 5 / kg.

- Tariki ya 26 Werurwe gukomeza: Gukomeza kugira umuriro mwinshi hanyuma bigatera imbere.

- 29 Werurwe: Amaraso ya periferique morphologie: guturika 92%; yazamuye transaminase na bilirubin.

- 2 Mata: Gutangira gufatwa, bivurwa na diazepam.

- 2 Mata (Umunsi wa 10): Yatangiye kuvura methylprednisolone muminsi 3.

- CRS reaction: Icyiciro cya 3, CRES: Icyiciro cya 3.

- Ku ya 8 Mata (Umunsi wa 16): Isuzuma ryamagufwa yerekana igabanuka ryuzuye rya morphologie, flux cytometrie mbi kubiturika bibi; MLL-ENL fusion gene isesengura ryinshi: 0.