Leukemia Lymphoblastic Acute (T-ALL)-01

Nā hiʻohiʻona lapaʻau:

- ʻIke: T-cell lymphoblastic lymphoma/leukemia

- Malaki 2020: Hāʻawi ʻia me ka ʻū paroxysmal a me ka mediastinal mass, i hoʻopaʻa ʻia ʻo T-cell lymphoblastic lymphoma e ka mediastinal mass puncture biopsy.

- Loaʻa iā 8 mau manawa o ka chemotherapy a ʻoi aku ma mua o 20 mau manawa o ka radiotherapy, ka hopena i ka hōʻemi nui o ka mediastinal mass.

- Ianuali 16, 2021: Hoʻoulu ʻia ka ʻeha ma ka ʻaoʻao lalo ʻākau.

- Ka maʻamau o ke koko: WBC 122.29 x 10^9/L, HGB 91 g/L, PLT 51 x 10^9/L

- Ka moʻo o ka iwi: 95.5% lymphoblasts kahiko.

- Cytometry kahe o ka iwi iwi: 91.77% o nā pūnaewele he lymphoblasts T-cell ʻaʻole.

- Ka hoʻokaʻina Genetic: ʻIke ʻia nā hoʻololi ʻana ma NOTCH1, IL7R, ASXL2 genes.

- Loaʻa iā Hyper-CVAD/B regimen, ESHAP regimen ma hope mai, ʻaʻole maikaʻi ʻelua me ke kuni mau.

- Pepeluali 18, 2021: Hoʻokomo ʻia i ko mākou haukapila.

- Hōʻike ʻia me ke kuni, hōʻike ka umauma CT i ka maʻi pneumonia.

- Ka maʻamau o ke koko: WBC 2.89 x 10^9/L, HGB 57.7 g/L, PLT 14.9 x 10^9/L

- ʻO ke koko ʻokoʻa ʻē aʻe: 90%

- Ka moʻo o ka iwi iwi: Hypercellular (IV grade), 85% primitive lymphoblasts.

- Immunophenotyping: 87.27% o nā pūnaewele he mau lymphoblasts T-cell kahiko.

- Ka nānā 'ana o Chromosomal: 46,XX [24]; ʻekolu mau karyotypes ʻē aʻe i ʻike ʻia.

- Nā genes i hoʻololi ʻia:

1. IL7R T244_I245insARCPL mutation maikai

2. NOTCH1 E1583_Q1584dup mutation maikaʻi

3. ASXL2 Q602R hoʻololi maikaʻi

- Ka nānā ʻana i ka maʻi leukemia fusion gene: ʻAʻohe

- Nā hualoaʻa PET/CT: ʻAʻohe mea koʻikoʻi hypermetabolic tumor foci i loko o ka iwi iwi āpau a me ka iwi iwi.

Lapaʻau:

- Hoʻomaka VP regimen chemotherapy, kikoʻī penei: Vincristine (VDS) 3mg hoʻokahi, Dexamethasone (Dex) 7mg i kēlā me kēia 12 hola no 9 mau lā, me ka mālama anti-infective.

- Malaki 1: Ua hoʻemi ʻia nā ʻōuli o ke koko ʻokoʻa i 7%.

- Malaki 4: ʻOhi ʻia nā lymphocytes autologous no ka moʻomeheu cell CD7-CAR T.

- Malaki 8: Hoʻomaka ka hoʻoponopono VLP i hui pū ʻia me ka mālama ʻana iā Sida benzamine.

- Malaki 14: Loaʻa iā FC regimen chemotherapy (Fludarabine 0.35g no nā lā 3, Cyclophosphamide 45mg no nā lā 3).

- Malaki 17 (infusion pre-cell):

- He immunophenotyping koena o ka iwi iwi: 15.14% mau keena e hōʻike ana i ka ʻālohilohi CD7, CD3 dim, cytoplasmic CD3, T cell receptor restricted delta (TCRrd), hōʻike hapa o CD99, e hōʻike ana i nā pūnaewele T mua loa.

- Malaki 19: Hoʻokomo ʻia nā pūnaewele CD7-CAR T autologous (1 x 10^6/kg).

- Nā hopena pili pili i ka CAR-T: Papa 1 CRS (ke kuni), ʻaʻohe neurotoxicity.

- ʻApelila 6 (La 17): Ua hōʻike ʻia ke kala ʻana o ka ʻāʻī o ka iwi, ʻaʻole i ʻike ka cytometry kahe i nā cell primitive malignant.