Ngā Kāwai Take

Take Whakaaturanga

Ko te Whakatairanga Ira Hou e tuku ana i te tumanako hou mo nga turoro o te mate pukupuku me te thalassemia

FUCASO: Ko te Whakatairanga Tino-tangata BCMA CAR-T Therapy me te kaha me te haumaru kore e rite.

Melanoma mata (i te tuatahi), ka whai mai ko nga pukupuku ate metastatic-02

Ko te taumata matatau IV mate pukupuku-01

Ko te mate pukupuku repeure teitei e haere tahi ana me nga metastases wheua maha-05

Acute Lymphoblastic Leukemia(B-ALL)-02

Te manawanui: Wang XX

Ira tangata: Wahine

Te tau: 3 tau

Nationality: Hainamana

Te tātaritanga:Acute Lymphoblastic Leukemia(B-KATOA)

Whakapā mai

Nga ahuatanga o te keehi:

- Mei 19, 2019: Kua mate te mate pukupuku B-cell lymphoblastic leukemia (B-KATOA)

- He maha nga papatipu mahunga me te lymphadenopathy

- Nga mahinga toto: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, lymphocytes rerekee 4%

- Te ahua o te kiko o te wheua: 80.2% nga lymphoblasts kore-pakeke (nga pupuhi)

- Immunophenotyping: 74.19% o ngā pūtau he pūtau tūroro B-lineage precursor e whakaatu ana i te CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, he wahanga cIgM. Tatautanga: B-KATOA (Waahi o mua-B)

- Ira whakakotahitanga: MLL-ENL pai, Philadelphia chromosome-rite (Ph-rite) mata kino

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

- I te tuatahi i tutuki i te tikanga whakamomori a VDLD te murunga mate mate i muri i te 1 marama, MLL-ENL ine PCR 0.026%

- Ka haere tonu te chemotherapy e ai ki te kawa tamariki, MLL-ENL ine PCR 0 i muri i te huringa tuawha. I haere tonu etahi atu chemotherapy.

- Poutū-te-rangi 2020: Ko te toenga o te mate pukupuku o te wheua wheua 0.35%, MLL-ENL ine PCR 0.53%, e tohu ana i te ahua o te hokinga mai. I whakaae te whanau ki te whakawhiti. Ka haere tonu te chemotherapy mo nga huringa e 3.

- Hōngongoi 2020: Kua hoki mai ano te hinu wheua.

- Noema 11, 2020: Intrathecal chemotherapy, CSF immunological toenga mate 66%, kua kitea he kano kano o te punaha nerve matua. E rua nga wa i tuaruatia ai te chemotherapy intrathecal, ka huri kino te CSF.

- Hakihea 31, 2020: I whakauruhia ki to maatau hohipera.

- Nga mahinga toto: WBC 3.99 x 10^9/L, HGB 66 g/L, PLT 57 x 10^9/L

- Tatau pupuhi toto tahataha: 69%

- Te ahua o te hinu wheua: 90% nga lymphoblasts kare i te pakeke (nga pupuhi)

- Immunophenotyping: 84.07% o nga pūtau e whakaatu ana i te CD38, te CD19, te CD81dim, te cCD79a, te HLA-DR, te cIgM, te CD22, te CD123, te whakaatu i te CD24, te CD15dim, e tohu ana i nga lymphoblasts B kore pakeke.

- Ira whakakotahitanga: MLL-ENL ira whakakotahitanga pai, PCR ine 44.419%

- Te ira ira: KMT2D te rerekee pai (te takenga o te germline)

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

- PET-CT: Te piki haere o te tinana i roto i te katoa o te koiwi me te kohao wheua wheua, te whakapae nui kei te hoki mai ano te reukemia; splenomegaly me te piki haere o te paopaotanga, ka uru pea ki te reukemia.

- I mahia te werohanga lumbar me te chemotherapy intrathecal kotahi, karekau he kino i kitea i roto i nga whakamatautau e pa ana ki te CSF.

Maimoatanga:

- E rua wiki o VLP chemotherapy, ka pupuhi te toto peripheral 5% i te Hanuere 18.

- Hanuere 25: He 91% te pupuhi toto i te taha, ka rongoatia ki te CTX, Ara-C, 6-MP chemotherapy.

- Hui-tanguru 3: Ka pupuhi te toto i te taha taha 22%.

- Hui-tanguru 4: Kohinga o te 50ml autologous toto peripheral mo CD19-CART ahurea pūtau.

- MTX 1g, FC chemotherapy (Rewharewha 15mg ia ra x 3 ra, CTX 0.12g ia ra x 3 ra).

- Hui-tanguru 13 (i mua i te whaowhia): E 87.5% te ahua o te ahua o te hinu wheua, 79.4% nga pupuhi kino.

- MLL-ENL fusion ira tātari ine: 42.639%.

- Hui-tanguru 14: Te whaowhia o nga pūtau CART i te horopeta 5 x 10^5/kg.

- Nga paanga kino e pa ana ki te CAR-T: Koeke 1 CRS (kirika), kaore he neurotoxicity.

- Ra 20 i muri i te whaowhia: Ko te paninga toto e whakaatu ana i te tipu o te puku, te wahanga o te pūtau CART 0.07%.

- Kare e whai hua te rongoa pūtau CART.

- Poutū-te-rangi 8, 2021: Nga mahi toto: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.

- Te pupuhi toto i te taha taha: 83%. Ko te toto peripheral autologous i kohia 60ml mo te CD19/CD22 ahurea pūtau CART rua.

- He rongoa ki te cytarabine me te dexamethasone hei whakahaere i te taumahatanga o te puku.

- Maehe 18: FC chemotherapy (Rewharewha 15mg ia ra x 3 ra, CTX 0.12g ia ra x 3 ra).

- Maehe 22 (i mua i te whaowhia): Nga mahi toto: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. Te ahua o te toto tahataha: 6% nga pupuhi.

- Te ahua o te hinu wheua: 91% nga pupuhi. Te toenga i roto i te hinu wheua: 88.61% nga pūtau e whakaatu ana i te CD38, CD19, cCD79a, CD81, CD22, e tohu ana i nga lymphoblasts B kore pakeke.

- MLL-ENL fusion ira tātari ine: 62.894%.

- Te tātari karyotype Chromosome: 46, XX, del(1)(p36.1), del(11)(q13), t(11;19)(q23;p13.3), tāpiri(14)(q34), - 17, +mar [2]/46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3 ), tāpiri(14)(q34).

- Poutū-te-rangi 23: Te whaowhia o nga pūtau CART i te horopeta 3 x 10^5/kg.

- Poutū-te-rangi 26 ki mua: Te kirika nui tonu me te whakawhanaketanga o te edema systemic.

- Poutū-te-rangi 29: Te ahua o te toto o te taha taha: 92% nga pupuhi; kua piki ake nga transaminases me te bilirubin.

- Paenga-whāwhā 2: Te tiimata o te hopu, ka rongoa ki te diazepam.

- Paenga-whāwhā 2 (Ra 10): I timata te maimoatanga methylprednisolone mo nga ra e 3.

- Tauhohenga CRS: Kōeke 3, CRES: Kōeke 3.

- Paenga-whāwhā 8 (Ra 16): Ko te arotakenga hinu wheua e whakaatu ana i te murunga o te ahua, te rere o te cytometry he kino mo nga pupuhi kino; MLL-ENL fusion ira tātari ine: 0.