ʻO ka maʻi Leukemia Lymphoblastic ʻoʻoleʻa (T-ALL)-01

Nā hiʻohiʻona lapaʻau:

- Hōʻike: lymphoma/leukemia lymphoblastic T-cell

- Malaki 2020: Hōʻike ʻia me ka ʻū paroxysmal a me ka puʻu mediastinal, i hōʻoia ʻia ka T-cell lymphoblastic lymphoma e ka biopsy puncture mediastinal mass.

- Ua loaʻa iā ia he 8 mau pōʻaiapuni o ka chemotherapy a me nā kau o 20 o ka radiotherapy, e hopena ana i ka emi nui ʻana o ka nui o ka mediastinal.

- Ianuali 16, 2021: Ua loaʻa ka ʻeha ma ka lālā ʻākau lalo.

- Ka papa hana koko: WBC 122.29 x 10^9/L, HGB 91 g/L, PLT 51 x 10^9/L

- ʻAno o ka iwi ʻiʻo: 95.5% nā lymphoblast mua.

- Ke kahe ʻana o ka iwi iwi: 91.77% o nā pūnaewele he mau lymphoblasts T-cell i ʻōpiopio.

- Ka hoʻokaʻina ʻana o nā ʻano hoʻoilina: Ua ʻike ʻia nā loli ʻana ma nā ʻano hoʻoilina NOTCH1, IL7R, ASXL2.

- Ua loaʻa i ka regimen Hyper-CVAD/B, a ma hope mai ua loaʻa ka regimen ESHAP, ʻaʻole i kūleʻa nā mea ʻelua me ke kuni mau.

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

- Hōʻike ʻia me ke kuni, ua hōʻike ka CT umauma i ka niumonia.

- Ka papa hana koko: WBC 2.89 x 10^9/L, HGB 57.7 g/L, PLT 14.9 x 10^9/L

- Nā pūnaewele koko ʻōpiopio peripheral: 90%

- ʻAno o ka iwi ʻiʻo: Hypercellular (papa IV), 85% primitive lymphoblasts.

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

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

- Nā genes i hoʻololi ʻia:

1. IL7R T244_I245insARCPL mutation maikaʻi

2. NOTCH1 E1583_Q1584dup mutation maikaʻi

3. Loaʻa ka hoʻololi ʻana o ASXL2 Q602R i kahi hopena maikaʻi

- Nānā ʻana i ka hui ʻana o ka leukemia: Maikaʻi ʻole

- Nā hopena PET/CT: ʻAʻohe foci tumor hypermetabolic koʻikoʻi i loko o ka lua iwi holoʻokoʻa a me ka iwi momona.

Lapaʻau:

- Ua hoʻomaka ka chemotherapy VP regimen, e like me kēia: Vincristine (VDS) 3mg hoʻokahi manawa, Dexamethasone (Dex) 7mg i kēlā me kēia 12 hola no 9 lā, me ka lāʻau anti-infective.

- Malaki 1: Ua hoʻemi ʻia nā hunaola koko ʻōpiopio i 7%.

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

- Malaki 8: Hoʻomaka ka regimen VLP i hui pū ʻia me ka lāʻau Sida benzamine.

- Malaki 14: Ua loaʻa iaʻu ka lāʻau lapaʻau FC regimen (Fludarabine 0.35g no 3 lā, Cyclophosphamide 45mg no 3 lā).

- Malaki 17 (hoʻokomo mua ʻana o ke kelepona):

- Immunophenotyping koena o ka iwi iwi: hōʻike ka 15.14% o nā pūnaewele i ka CD7 ʻālohilohi, CD3 dim, cytoplasmic CD3, T cell receptor restricted delta (TCRrd), hōʻike hapa o CD99, e hōʻike ana i nā pūnaewele T primitive malignant.

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

- Nā hopena ʻaoʻao e pili ana iā CAR-T: Papa 1 CRS (kuni), ʻaʻohe neurotoxicity.

- ʻApelila 6 (Lā 17): Ua hōʻike ke ʻano o ka iwi iwi i ke kala ʻana, ʻaʻole i ʻike ka cytometry kahe i nā pūnaewele primitive malignant.