ʻO ka maʻi leukemia lymphoblastic koʻikoʻi (B-ALL)-03

Nā Hiʻohiʻona Hihia:

- Ua maʻi ʻia me ka leukemia lymphoblastic B-cell acute i ka hopena o Mei 2020.

- Ka papa hana koko: WBC 5.14x10^9/L, HGB 101.60g/L, PLT 6x10^9/L.

- ʻAno o ka iwi ʻiʻo: Hypocellular me 67% mau lymphocytes primitive.

- Ka cytometry kahe: 82.28% o nā pūnaewele e hōʻike ana iā CD38, HLA-DR, CD19, CD10, CD105, TDT, CD22, cCD79a, hōʻike hapa ʻia ʻo CD9, hōʻike nāwaliwali ʻia ʻo CD13.

- ʻAʻole maikaʻi ka nānā ʻana o ka gene fusion; WT1 57.3%; ʻaʻohe gene fusion e pili ana i ka PH-like ALL i ʻike ʻia.

- IʻA: maikaʻi ka loli ʻana o TP53.

- Nā Chromosome: 63-58, XXY, +Y, +1, +del(1)(q41q42), -2, -3, +6, -7, +8, -9, +10, -12, -13, +14, +15, -17, +18, -20, +22(cp16)/46, XY[4].

- Ua loaʻa i ka regimen VCDLP no 2 mau papa me ke kala ʻole.

- ʻO ka regimen CAM-VL (CTX 2gx2, Arac 200mgx6, 6-MP 100mgx14, VDS 4mgx2, L-ASP 10,000 IUx7) ma Iulai 22, 2020, ʻaʻole naʻe i hoʻōla ʻia.

- ʻO ke kahe ʻana o ka iwi iwi ma ka lā 25 o Kepakemapa, 2020: 7.35% o nā pūnaewele e hōʻike ana iā CD81, CD19, CD10, CD38, CD33, hōʻike nāwaliwali iā CD20, CD45.

- Ka nānā ʻana i ka hoʻololi ʻana o ka puʻupuʻu koko: ka hoʻololi ʻana o TP53.

- Nā Chromosome: 46, XY[20].

- Ua hoʻomaka i ka lāʻau lapaʻau CD19-CART.

- Ka papa hana FC (FLU 62.7mg x 4 lā, CTX 1045mg x 2 lā) chemotherapy.

- ʻOkakopa 1, 2020: Hoʻokomo ʻia ʻana o nā pūnaewele CD19-CART Autologous ma 4.7x10^7/kg.

- CRS papa 2 me ka neurotoxicity papa 1, i hoʻomaikaʻi ʻia ma hope o ka mālama kākoʻo.

- ʻOkakopa 29, 2020: Hoʻopau piha ʻia ke ʻano o ka iwi ʻiʻo, ʻaʻohe mau pūnaewele primitive malignant ma ka cytometry kahe.

- Kekemapa 31, 2020: ʻŪhū maloʻo, nausea, luaʻi, nāwaliwali ākea.

- Ka papa hana koko: WBC 15.53x10^9/L, HGB 134g/L, PLT 71x10^9/L.

- Ka ʻūmiʻi ʻana o ka iwi e hōʻike ana i ka hoʻi hou ʻana.

- Ianuali 2, 2021: Ua hoʻokomo ʻia i ko mākou haukapila.

- Ka papa hana koko: WBC 20.87x10^9/L, HGB 118.30g/L, PLT 58.60x10^9/L.

- Creatinine 134umol/L, ke koko kiʻekiʻe pae 3, mōʻaukala olakino o 4 mau makahiki.

- Ka hoʻokaʻawale ʻana o ke koko peripheral: 62% nā cell primitive.

- Immunophenotyping: 28.48% o nā cell (nā cell nucleated) e hōʻike ana i ka CD10, CD38dim, HLA-DR, CD20dim, CD24, CD81, cCD79a, CD22, CD268dim, CD58, hōʻike hapa ʻia ka CD123, TDT, ʻaʻole hōʻike i ka CD34, CD19, MPO, CD117, CD13, CD33, CD11b, clgM, CD79b, CD7, cCD3, kappa, lambda, e hōʻike ana i nā lymphocytes B primitive malignant.

- Ka nānā ʻana i ka hoʻololi ʻana o ka puʻupuʻu koko: maikaʻi ka hoʻololi ʻana o TP53 R196P.

Lapaʻau:

- Ua loaʻa iā ia ka chemotherapy VLP, me ka mālama ʻana no ke koko kiʻekiʻe, ka hoʻemi ʻana o ka creatinine, a me ka hydration alkalinization.

- Ianuali 19: Ua hōʻike ka papa hana koko i ka WBC 1.77x10^9/L, HGB 71g/L, PLT 29.8x10^9/L.

- Ka hoʻokaʻawale ʻana o ke koko peripheral: ʻAʻohe lymphocytes primitive.

- ʻAno o ka iwi ʻiʻo: Hypercellularity (papa V), nā wahi kikowaena o ka papa IV, me 42% o nā lymphocytes primitive.

- Ka cytometry kahe: 13.91% o nā pūnaewele e hōʻike ana iā CD10, cCD79a, CD38, CD81, CD22, ʻaʻole hōʻike iā CD20, CD34, CD19, e hōʻike ana i nā pūnaewele B mua ʻino.

- Kariotio Chromosomal:

- 35,XY,-2,-3,-4,-5,-7,-9,-12,-13,-16,-17,-20[8]/35,XY,+X,-2,-3,-4,-5,-7,-9,-10,-12,-13,-16,-17,-20[1]/36,XY,hoʻohui(1)(q42),-2,-3,-4,-7,-9,-12,-13,-16,-17,-20[1]/46,XY[20].

- Ianuali 20: Ua hōʻiliʻili ʻia nā lymphocytes no ka moʻomeheu pūnaewele CD22-CART.

- Ianuali 21: Hana ʻia ka puncture lumbar, lawelawe ʻia ka chemotherapy intrathecal e pale aku i ka leukemia o ka ʻōnaehana nerve waena; ʻaʻohe mea ʻē aʻe i hōʻike ʻia ma ka hoʻokolokolo ʻana o ka wai cerebrospinal.

- Ianuali 22: Ua loaʻa iā Arac, 6MP, L-ASP chemotherapy, a me FC (Flu 50mg x 3, CTX 0.5gx 3) chemotherapy.

- Pepeluali 7 (ma mua o ka hoʻokomo ʻana): Ua hōʻike ke ʻano o ka iwi ʻiʻo i ka 93% o nā lymphocytes primitive.

- Ka cytometry kahe: 76.42% o nā pūnaewele e hōʻike ana iā CD38, cCD79a, CD22, cbcl-2, CD123, CD10bri, CD24, CD81, ʻaʻole hōʻike iā CD4, CD3, CD13+33, CD34, CD20, CD19, CD279 (PD1), CD274 (PDL1), e hōʻike ana i nā pūnaewele B mua ʻino.

- Ua ulu aʻe ka maʻi ʻili a me nā ʻiʻo palupalu me ke kuni; ua hoʻomaikaʻi ʻia ma hope o ka lāʻau antibiotic.

- Pepeluali 9: Hoʻokomo ʻia ʻana o nā pūnaewele CD22-CART Autologous (5x10^5/kg).

- Nā hopena ʻaoʻao e pili ana iā CAR-T: CRS grade 1, kuni ma ka Lā 6 me Tmax 40°C, mahana i kāohi ʻia ma ka Lā 10; ʻaʻohe neurotoxicity.

- Malaki 11: Ua hōʻike ka loiloi ʻana o ka iwi iwi i ke kala piha ʻana o ke ʻano, ʻaʻole i hōʻike ka cytometry kahe i nā pūnaewele primitive malignant.