Te Mate Pūkahukahu Lymphoblastic Whakapeka (B-ALL)-01

I kitea he mate pukupuku toto pūtau-B acute i te 28 o Noema, 2017.

Te maimoatanga VDLP i te tīmatanga, kia eke ki te murunga hapa o te hinu wheua (kāore anō kia pūrongohia ngā taipitopito).

Hui-tanguru 2018: I huri ki te tikanga VLCAM. I whakaatuhia e te inenga rere o te hinu wheua he 60.13% o ngā pūtau B kino kāore anō kia pakari.

Maehe 2018: I whakaurua ki te whakamātautau haumanu BiTE. Te murunga āhua i roto i te hinu wheua, kāore he pūtau kino kāore anō kia pakari i kitea e te flow cytometry.

8 Mei, 2018: I whiwhi i te maimoatanga whakapakari TBI/CY+VP16, ā, i muri mai ka whakatōkia ngā pūtau take allogeneic mai i te tuakana/teina kua tino ōrite (te kaituku AB+ ki te kaiwhiwhi A+). Te whakaoranga o ngā neutrophil i te rā +11, te whakaoranga o ngā megakaryocyte i te rā +12.

Hakihea 5, 2018: Kua tino murua te āhua o te hinu wheua, kāore he pūtau kino kāore anō kia pakari i kitea e te flow cytometry. I whiwhi i te werohanga lymphocyte kaituku (DLI) me te maimoatanga ārai mate me te dasatinib me te imatinib hei ārai i te hokinga mai.

2 o Pēpuere, 2019: I whakaatuhia e te āhua o ngā pūtau kāore anō kia pakari te 6.5%, i whakaatuhia e te ine rerenga pūtau te 0.08% o ngā pūtau pūtau kino kāore anō kia pakari te B lymphoblasts. I whiwhi i te rongoā DLI. 28 o Poutū-te-rangi, 2019: Kāore i whakaatuhia e te ine rerenga pūtau ngā āhuatanga rerekē.

11 o Ākuhata, 2019: Ka hoki anō te mate o te hinu wheua, i rongoatia ki te dasatinib.

2 o Hepetema, 2019: I whakaatuhia e te āhua o te pūtau he 3% kāore anō kia pakari, i whakaatuhia e te inenga rere o te pūtau he 0.04% ngā pūtau kino kāore anō kia pakari. I haere tonu te maimoatanga ki te dasatinib, ā, i muri mai ka rua ngā huringa o te rongoā chemotherapy methotrexate.

11 Mei 2020: Ka hoki anō te hinu wheua ki te mate.

I te tau 2020 i whiwhi ahau i ngā maimoatanga pūtau CD19-CAR-T e rua me ngā maimoatanga pūtau CD19-CAR-T allogeneic e rua, engari kāore i tutuki tetahi o aua maimoatanga.

26 o Oketopa, 2020: I whakaurua ki tō mātou hōhipera.

Ngā Kitenga Taiwhanga:

Te mahinga toto: WBC 22.75 x 10^9/L, HGB 132 g/L, PLT 36 x 10^9/L

Ngā pūtau toto ā-taha kāore anō kia pakari: 63%

Te āhua o te hinu wheua: Ngā pūtau nui rawa (kōeke II), 96% ngā pūtau toto iti kāore anō kia pakari.

Te Tātaritanga Ārai Mate: Ka whakaatuhia e ngā pūtau te CD19, cCD79a, CD38dim, CD10bri, CD34, CD81dim, CD24, HLA-DR, TDT, CD22, CD72; te whakaaturanga hapa o te CD123. Kua tautuhia hei pūtau pūtau pūtau kino kāore anō kia pakari.

Ngā whakarerekētanga o te pukupuku toto: Kāore i te pai.

Ira hanumi mate pukupuku toto: He pai te ira hanumi NUP214-ABL1.

Tātaritanga ira: 46, XX, t(1;9)(p34;p24), tāpiri(11)(q23)[4]/46, XX, t(1;9)(p34;p24), tāpiri(11)(q23)x2 [2]/46, XX[3]

Kimerisma: Ko ngā pūtau i ahu mai i te kaituku ko te 7.71%.

Te maimoatanga:

- Kua tukuna te tikanga rongoā rongoā VDS, DEX, LASP.

- 20 Whiringa-ā-rangi: Ngā pūtau toto ā-taha kāore anō kia pakari 0%.

- Kohikohi i ngā pūtau toto ā-taha autologous mō te ahurea pūtau CAR-T takirua CD19/22.

- 29 o Noema: Te rongoā mate pukupuku FC (Rewharewha 50mg x 3, CTX 0.4gx 3).

- 2 o Hakihea (i mua i te werohanga o ngā pūtau CAR-T):

- Te whakamātautau toto: WBC 0.44 x 10^9/L, HGB 66 g/L, PLT 33 x 10^9/L.

- Te āhua o te hinu wheua: Ngā pūtau nui rawa (kōeke IV), 68% ngā pūtau toto iti kāore i te pakari.

- Aromatawai tauanga o te ira honohono NUP214-ABL1: 24.542%.

- Te tātaritanga rere o te pūtau: 46.31% o ngā pūtau e whakaatu ana i te CD38dim, CD22, BCL-2, CD19, CD10bri, CD34, CD81dim, CD24, cCD79a, e tohu ana i ngā pūtau pūtau kino kāore anō kia pakari.

- Hakihea 4: Te werohanga o ngā pūtau CAR-T takirua CD19/22 autologous (3 x 10^5/kg).

- Ngā pānga taha e pā ana ki te CAR-T: CRS Tau 1, kirika i te Rā 6 me te Tmax o te 40°C, kirika kua whakahaeretia i te Rā 10. Kāore i kitea he paitini roro.

- Hakihea 22 (Aromatawai Rā 18): Te murunga katoa o te āhua i roto i te hinu wheua, kāore he pūtau kino kāore anō kia pakari i kitea e te ine rere. Aromatawai tauanga o te ira whakakotahitanga NUP214-ABL1: 0%.