ʻO ka maʻi leukemia lymphoblastic koʻikoʻi (B-ALL)-02

Nā ʻano hihia:

- Mei 19, 2019: Ua ʻike ʻia me ka leukemia lymphoblastic B-cell acute (B-ALL)

- Hōʻike ʻia me nā puʻupuʻu poʻo he nui a me ka lymphadenopathy

- Ka papa hana koko: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, nā lymphocytes ʻano ʻē 4%

- ʻAno o ka iwi ʻiʻo: 80.2% lymphoblasts ʻōpiopio (blasts)

- Immunophenotyping: 74.19% o nā cell he mau cell precursor B-lineage malignant e hōʻike ana iā CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, e hōʻike hapa ana iā cIgM. Hōʻike: B-ALL (kahua Pre-B)

- Kine Fusion: MLL-ENL maikaʻi, Philadelphia chromosome-like (Ph-like) pale maikaʻi ʻole

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

- Ua hoʻokō mua ka chemotherapy VDLD i ka hoʻokuʻu ʻana o ka immunological ma hope o 1 mahina, MLL-ENL quantitative PCR 0.026%

- Hoʻomau ʻia ka chemotherapy e like me ke kaʻina hana pediatric, MLL-ENL quantitative PCR 0 ma hope o ka pōʻaiapuni 4. Ua hoʻomau ʻia ka chemotherapy hou aʻe.

- Malaki 2020: ʻO ka maʻi koena immunological iwi 0.35%, MLL-ENL quantitative PCR 0.53%, e hōʻike ana i kahi ʻano e hoʻi hou ai. Ua hōʻole ka ʻohana i ka hoʻoili ʻana. Hoʻomau ka chemotherapy no 3 mau pōʻaiapuni.

- Iulai 2020: Ua hoʻi hou ka iwi iwi me ka piha.

- Nowemapa 11, 2020: Intrathecal chemotherapy, CSF immunological residual disease 66%, i ʻike ʻia me ka leukemia o ka ʻōnaehana nerve waena. Ua hana hou ʻia ka chemotherapy intrathecal i ʻelua manawa, ua lilo ka CSF i mea maikaʻi ʻole.

- Kekemapa 31, 2020: Ua hoʻokomo ʻia i ko mākou haukapila.

- Ka papa hana koko: WBC 3.99 x 10^9/L, HGB 66 g/L, PLT 57 x 10^9/L

- Ka helu o ka pahū koko peripheral: 69%

- ʻAno o ka iwi ʻōʻō: 90% lymphoblasts ʻōpiopio (blasts)

- Immunophenotyping: 84.07% o nā pūnaewele e hōʻike ana iā CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, hōʻike hapa iā CD24, CD15dim, e hōʻike ana i nā lymphoblasts B ʻōpiopio malignant.

- Kine Fusion: MLL-ENL fusion gene maikaʻi, quantitative PCR 44.419%

- Hoʻololi ʻana o ka ʻano hoʻoilina: maikaʻi ka hoʻololi ʻana o KMT2D (kumu germline)

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

- PET-CT: Hoʻonui ka metabolic pālahalaha ma ka lua iwi holoʻokoʻa a me ka iwi, manaʻo nui ʻia ka hoʻi hou ʻana o ka leukemia; splenomegaly me ka hoʻonui ʻana i ka metabolism, e pili ana paha i ka leukemia.

- Hana ʻia ka lumbar puncture a me ka intrathecal chemotherapy i hoʻokahi manawa, ʻaʻohe ʻano ʻē i loaʻa i nā hoʻokolohua e pili ana i ka CSF.

Lapaʻau:

- ʻElua pule o ka chemotherapy VLP, nā pahū koko peripheral 5% ma Ianuali 18.

- Ianuali 25: Ua piʻi aʻe ke koko peripheral i 91%, ua mālama ʻia me CTX, Ara-C, 6-MP chemotherapy.

- Pepeluali 3: Pahū koko peripheral 22%.

- Pepeluali 4: Hōʻiliʻili ʻana o 50ml koko peripheral autologous no ka moʻomeheu cell CD19-CART.

- MTX 1g, FC chemotherapy (Flu 15mg i kēlā me kēia lā x 3 lā, CTX 0.12g i kēlā me kēia lā x 3 lā).

- Pepeluali 13 (ma mua o ka hoʻokomo ʻana): Hōʻike ke ʻano o ka iwi iwi i 87.5% mau pahū, hōʻike ka cytometry kahe i 79.4% mau pahū ʻino.

- Ka loiloi helu o ka hui ʻana o ka gene MLL-ENL: 42.639%.

- Pepeluali 14: Hoʻokomo ʻia ʻana o nā pūnaewele CART ma kahi mahele o 5 x 10^5/kg.

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

- Lā 20 ma hope o ka hoʻokomo ʻana: Hōʻike ka paʻi koko i ka ulu ʻana o ka puʻupuʻu, ka nui o nā cell CART 0.07%.

- Ka lāʻau lapaʻau CART cell maikaʻi ʻole.

- Malaki 8, 2021: Ka hana koko: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.

- Nā pahū koko peripheral: 83%. Ua hōʻiliʻili ʻia ke koko peripheral autologous 60ml no ka moʻomeheu cell CD19/CD22 pālua CART.

- Hoʻohana ʻia ka cytarabine a me ka dexamethasone e kāohi i ka nui o ka maʻi puʻupuʻu.

- Malaki 18: FC chemotherapy (Flu 15mg i kēlā me kēia lā x 3 lā, CTX 0.12g i kēlā me kēia lā x 3 lā).

- Malaki 22 (ma mua o ka hoʻokomo ʻana): Ka hana koko: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. ʻAno koko peripheral: 6% blasts.

- ʻAno o ka iwi ʻiʻo: 91% pahū. Koena i loko o ka iwi ʻiʻo: 88.61% mau pūnaewele e hōʻike ana iā CD38, CD19, cCD79a, CD81, CD22, e hōʻike ana i nā lymphoblast B ʻōpiopio malignant.

- Ka loiloi helu o ka hui ʻana o ka gene MLL-ENL: 62.894%.

- Ka nānā ʻana i ke karyotype chromosome: 46, XX, del(1)(p36.1), del(11)(q13), t(11;19)(q23;p13.3), add(14)(q34), -17, +mar [2]/46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3), add(14)(q34).

- Malaki 23: Hoʻokomo ʻia ʻana o nā pūnaewele CART ma kahi mahele o 3 x 10^5/kg.

- Malaki 26 a ma hope: Ke kuni nui mau nei a ma hope iho ua loaʻa ka edema systemic.

- Malaki 29: ʻAno koko peripheral: 92% pahū; kiʻekiʻe nā transaminases a me bilirubin.

- ʻApelila 2: Hoʻomaka nā maʻi ʻōʻili, mālama ʻia me ka diazepam.

- ʻApelila 2 (Lā 10): Hoʻomaka i ka lāʻau methylprednisolone no 3 mau lā.

- Ka hopena CRS: Papa 3, CRES: Papa 3.

- ʻApelila 8 (Lā 16): Hōʻike ka loiloi ʻana o ka iwi iwi i ka hoʻomaha piha ʻana o ke ʻano, ʻaʻole maikaʻi ka cytometry kahe no nā pahū malignant; MLL-ENL fusion gene quantitative analysis: 0.