Leukemia Lymphoblastic Acute (B-ALL)-02

Nā ʻano hiʻohiʻona:

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

- Hāʻawi ʻia me ka nui o ke poʻo a me ka lymphadenopathy

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

- Ka moʻo ʻana o ka iwi iwi: 80.2% nā lymphoblast ʻaʻole oʻo (pahū)

- Immunophenotyping: 74.19% o nā pūnaewele he malignant B-lineage precursor cell e hōʻike ana i ka CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, hōʻike hapa cIgM. ʻIke: B-ALL (Pre-B pae)

- Gene Fusion: MLL-ENL maikaʻi, Philadelphia chromosome-like (Ph-like) pale ʻino

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

- VDLD regimen chemotherapy i loaʻa mua i ke kala immunological ma hope o 1 mahina, MLL-ENL quantitative PCR 0.026%

- Hoʻomau ka chemotherapy e like me ka protocol pediatric, MLL-ENL quantitative PCR 0 ma hope o ka pōʻai 4. Ua hoʻomau hou ʻia ka chemotherapy.

- Malaki 2020: ʻO ke koena o ka maʻi maʻi iwi iwi 0.35%, MLL-ENL quantitative PCR 0.53%, e hōʻike ana i ke ʻano o ka hoʻi hou ʻana. Ua hōʻole ka ʻohana i ka hoʻololi ʻana. Hoʻomau ʻia ka chemotherapy no 3 mau manawa.

- Iulai 2020: Ua hoʻi hou ka iwi iwi.

- Nowemapa 11, 2020: Intrathecal chemotherapy, CSF immunological koena maʻi 66%, i ʻike ʻia me ka maʻi leukemia ʻōnaehana waena. Ua hana hou ʻia ka chemotherapy Intrathecal, ua huli hewa ʻo CSF.

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

- Nā hana maʻamau o ke koko: WBC 3.99 x 10^9/L, HGB 66 g/L, PLT 57 x 10^9/L

- Ka helu ʻana o ke koko ʻaoʻao: 69%

- Ka hoʻonaʻauao ʻana o ka iwi iwi: 90% lymphoblasts oʻo ʻole (pahū)

- Immunophenotyping: 84.07% o nā pūnaewele hōʻike CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, hōʻike hapa CD24, CD15dim, e hōʻike ana i nā lymphoblast B ʻaʻole i oʻo.

- Gene hoʻohui: MLL-ENL fusion gene maikaʻi, PCR nui 44.419%

- Hoʻololi Genetic: KMT2D mutation maikaʻi (germline kumu)

- Karyotype 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: Ka hoʻonui ʻana i ka metabolic i loko o ka iwi iwi a me ka iwi iwi, ke kānalua nui o ka hoʻi hou ʻana o ka maʻi leukemia; splenomegaly me ka hoʻonui ʻana i ka metabolism, pili paha i ka maʻi leukemia.

- Hana ʻia ka lumbar puncture a me ka chemotherapy intrathecal i hoʻokahi manawa, ʻaʻohe mea ʻino i loaʻa i nā hoʻokolohua pili i ka CSF.

Lapaʻau:

- ʻElua mau pule o ka hoʻomaʻamaʻa ʻana o ka VLP, hoʻoheheʻe ʻia ke koko peripheral 5% ma Ianuali 18.

- Ianuali 25: He 91% ke kahe o ke koko pili, mālama ʻia me CTX, Ara-C, 6-MP chemotherapy.

- Fepeluali 3: He 22%.

- Feberuari 4: Ohi o 50ml autologous peripheral koko no CD19-CART moʻomeheu cell.

- 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).

- Feberuari 13 (pre-infusion): He 87.5% ka pahū ʻana o ka iwi iwi, a he 79.4% ka pahū ʻino.

- MLL-ENL fusion gene quantitative analysis: 42.639%.

- Feberuari 14: Infusion o nā pūnaewele CART ma ke ʻano o 5 x 10^5/kg.

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

- La 20 ma hope o ka hoʻokomo ʻana: Hōʻike ka hamo koko i ka hoʻonui ʻia ʻana o ka maʻi maʻi, CART cell proportion 0.07%.

- ʻAʻole pono ka CART cell therapy.

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

- Pahū koko ʻaoʻao: 83%. Ua hōʻiliʻili ʻia ke koko peripheral autologous he 60ml no ka moʻomeheu cell CART ʻelua CD19/CD22.

- Hoʻohana ʻia me ka cytarabine a me ka dexamethasone e hoʻomalu i ke kaumaha o ka maʻi maʻi.

- 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 (pre-infusion): Nā hana maʻamau o ke koko: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. ʻO ke koko ʻokoʻa: 6% pahū.

- ʻO ke kino iwi iwi: 91% pahū. Ke koena i loko o ka iwi iwi: 88.61% mau keena e hōʻike ana i ka CD38, CD19, cCD79a, CD81, CD22, e hōʻike ana i nā lymphoblast B ʻaʻole i oʻo.

- MLL-ENL fusion gene quantitative analysis: 62.894%.

- Ka nānā 'ana 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 ), hoʻohui(14)(q34).

- Malaki 23: Infusion o nā pūnaewele CART ma ka nui o 3 x 10^5/kg.

- Malaki 26 ma mua aku: ʻO ke kuni kiʻekiʻe mau a ma hope iho ua hoʻomohala ʻia ka edema systemic.

- Malaki 29: ʻO ke koko ʻokoʻa: 92% pahū; ʻO nā transaminase kiʻekiʻe a me bilirubin.

- ʻApelila 2: Ka hoʻomaka ʻana o ka hopu ʻana, mālama ʻia me ka diazepam.

- ʻApelila 2 (La 10): Hoʻomaka i ka mālama ʻana i ka methylprednisolone no 3 mau lā.

- Pane CRS: Papa 3, CRES: Papa 3.

- ʻApelila 8 (La 16): Hōʻike ka loiloi ʻana i ka ʻiʻo o ka iwi i ke kala piha ʻana o ka morphological, kahe cytometry maikaʻi ʻole no nā pahū ʻino; MLL-ENL fusion gene quantitative analysis: 0.