Leukemia ikabije ya Lymphoblastic (T-ALL)-02

Ibiranga ubuvuzi:

- Isuzuma: Leukemia y'urumogi ya T-cell lymphoblastic ikabije

- Itangira: Mu mpera za Werurwe 2018

- Ibimenyetso by'ibanze: Kwaguka kw'uturemangingo tw'umurabyo mu mubiri wose

- Gahunda y'amaraso ya mbere: WBC: 39.46*10^9/L, Hb: 129g/L, PLT: 77*10^9/L

- Imiterere y'umusemburo w'amagufwa: 92% by'ibisasu

-Uburyo bwo gupima imiterere y'uturemangingo: 95.3% by'uturemangingo tudasanzwe tugaragaza

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

- Utunyangingo tw’ingirabuzimafatizo: Negative

- Impinduka mu ngirabuzimafatizo: Impinduka mu ngirabuzimafatizo ya NOTCH1 yabonetse

- Isesengura rya chromosome: Karyotype isanzwe

Amateka y'ubuvuzi:

- 3 Mata 2018: Uburyo bwo kuvura hakoreshejwe uburyo bwa VDCP

- Ku ya 18 Mata 2018: Udukoko two mu magufwa twagize 96%

- 20 Mata 2018: Yagabanutse nyuma yo kugabanya gahunda ya CAG

- 18 Gicurasi 2018: Uburyo bwo kuvura hakoreshejwe uburyo bwa CMG + VP

- 22 Kamena 2018: Ubwinshi bw'amagufwa mu mitsi bwariyongereyeho 40%, indwara ya kanseri y'amaraso yongera kugaragara

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

- Gutera uturemangingo tw’amaraso dukomoka kuri HLA-matched sibling hakoreshejwe FLU+BU conditioning ku ya 14 Kanama

- Gukurikirana nyuma yo guterwa: Igabanuka ry'imiterere y'umusemburo w'amagufwa ku kwezi 1, amezi 3, amezi 6, amezi 9, n'amezi 11.

- Imiterere y'umusemburo w'amagufwa yagaragaje koroherwa nyuma y'amezi 16 nyuma yo guterwa, aho flow cytometry yagaragaje 0.02% by'ama-lymphocyte adakuze nabi.

- 13 Ugushyingo 2020: Kugabanya amaraso mu buryo bwa "peripheral blood chimerism" byaturutse ku baterankunga byari 97.9%

- Uturemangingo tw’amaraso yo mu mpande: 20%

- Ukuboza 18, 2020: Imiterere y'umusemburo w'amagufwa: 60.6% by'ibisebe

- Ingano y'amazi: 30.85% bya limfozeti za T zikiri mbi zitarakura

- Isesengura rya chromosome: 46, XY (20)

- Yahawe imiti igabanya ubukana bwa DA ku ya 19 Mutarama 2021

- Imiterere y'umusemburo w'amagufwa ku ya 19 Mutarama 2021: Hyperplasia yo mu cyiciro cya gatatu, 16% by'ibitero

- Isesengura rya karyotype ya chromosome: 46, XY (20)

- Ingano y'amaraso mu mubiri: 7.27% by'uturemangingo (muri uturemangingo twa nuclear) twagaragayemo CD99bri, CD13, CD38, cbcl-2, cCD3, HLA-ABC bri, CD7bri, na CD5dim yagaragayemo igice, bigaragaza lymphocytes T zitarakura neza.

- Gusuzuma imiterere y'uturemangingo twa kanseri y'amaraso: Negative

- Isesengura ry'impinduka mu maraso (ubwoko 86):

1. PHF6 K299Efs*13 yanduye mutation

2. RUNX1 S322* yanduye impinduka mu mubiri

3. FBXW7 E471G yanduye mutation

4. JAK3 M511I yanduye impinduka mu mubiri

5. NOTCH1 Q2393* yanduye impinduka mu mubiri

Uburyo bwo kuvura:

- 22 Mutarama: Gukusanya no gukusanya lymphocytes zo mu maraso zikomoka ku maraso zikoreshwa mu buryo bwa autologous kugira ngo CD7-CART ikoreshwe

- Mbere yo guterwa CD7-CART, umurwayi yahawe VLP (vincristine, l-asparaginase, prednisone) hamwe na chemotherapy ya bortezomib.

- Ku ya 3 Gashyantare: Imiti ya kanseri yo mu bwoko bwa FC (ibicurane 50mg mu gihe cy'iminsi 3 + CTX 0.45g mu gihe cy'iminsi 3)

- Ku ya 5 Gashyantare (mbere yo guterwa): Imiterere y'umusemburo w'amagufwa yagaragaje 23% by'ibisasu.

- Ingano y'amaraso mu mubiri yagaragaje 4.05% by'uturemangingo dufite CD99bri, CD5dim, CD7bri, TDT, cCD3, bigaragaza ko lymphocytes T zitarakura neza.

- Isesengura rya chromosome: 46, XY (20)

- Isesengura rya Chimerism (nyuma ya HSCT): Uturemangingo dukomoka ku batanga serivisi twagize 52.19%.

- Ku ya 7 Gashyantare: Gutera uturemangingo twa CD7-CART ku gipimo cya 5 * 10 ^ 5 / kg.

- 15 Gashyantare: Uturemangingo tw’amaraso yo mu mpande two mu maraso tudakuze twagabanutseho 2%.

- Ku ya 19 Gashyantare (Umunsi wa 12 nyuma yo guterwa): Umurwayi yagize umuriro, wamaze iminsi 5 mbere y'uko hagenzurwa ubushyuhe.

- Ku ya 2 Werurwe: Isuzuma ry'umusemburo w'amagufwa ryagaragaje ko habayeho kugabanuka k'imiterere y'umubiri, aho flow cytometry itagaragazaga uturemangingo twanduye tutaragera mu myaka.