Te Mate Pūkahukahu Lymphoblastic Whakapeka (B-ALL)-02

Ngā Āhuatanga o te Take:

- 19 Mei, 2019: I kitea he mate toto pūkahukahu pūtau-B (B-ALL)

- He maha ngā puranga o te tumuaki me te lymphadenopathy e puta mai ana

- Te whakamātautau toto: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, lymphocytes rerekē 4%

- Te āhua o te hinu wheua: 80.2% ngā pūtau toto iti (ngā pūtau toto iti)

- Te Tātaritanga Ārai Mate: 74.19% o ngā pūtau he pūtau matua o te whakapapa-B kino e whakaatu ana i te CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, e whakaatu ana i te cIgM. Tātaritanga: B-ALL (Wāhanga i mua i te B)

- Ira honohono: MLL-ENL pai, mata tirotiro kino rite ki te ira-Ph (rite ki te ira-Ph)

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

- I te tīmatanga, i tutuki i te rongoā chemotherapy VDLD te murunga ā-pūnaha i muri i te 1 marama, MLL-ENL quantitative PCR 0.026%

- I haere tonu te rongoā chemotherapy e ai ki te tikanga tamariki, MLL-ENL quantitative PCR 0 i muri i te huringa tuawhā. I haere tonu te rongoā chemotherapy anō.

- Maehe 2020: Mate toenga ārai mate o te hinu wheua 0.35%, PCR rahinga MLL-ENL 0.53%, e tohu ana i te ia o te hokinga mai. I whakakahoretia e te whānau te whakawhitinga. I haere tonu te chemotherapy mō ngā huringa e 3.

- Hōngongoi 2020: I hoki anō te hinu wheua ki tōna āhua tūturu.

- 11 o Noema, 2020: Te rongoā matepukupuku intrathecal, 66% te mate toenga ārai mate CSF, i kitea he mate toto totohu o te pūnaha io matua. E rua ngā wā i whakahokia ai te rongoā matepukupuku intrathecal, ā, i puta te CSF he kino.

- 31 o Hakihea, 2020: I whakaurua ki tō mātou hōhipera.

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

- Te maha o ngā pahūtanga toto ā-taha: 69%

- Te āhua o te hinu wheua: 90% ngā pūtau toto iti (ngā pūtau pupuhi)

- Te Tātaritanga Ārai Mate: 84.07% o ngā pūtau e whakaatu ana i te CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, e whakaatu ana i te CD24, CD15dim, e tohu ana i ngā pūtau pūtau kino kāore anō kia pakari.

- Ira honohono: He pai te hononga ira MLL-ENL, he PCR tauanga 44.419%

- Ngā whakarerekētanga ira: He pai te whakarerekētanga KMT2D (takenga mai i te ira tangata)

- Te momo ira tangata: 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: He pikinga horapa o te pākia o te tinana puta noa i te kōhao angaiwi me te hinu wheua, he nui te whakapae kei te hoki mai anō te mate toto; te splenomegaly me te pikinga o te pākia o te tinana, tera pea kei roto ko te mate toto.

- I mahia te pokanga lumbar me te chemotherapy intrathecal kotahi, kāore i kitea he rerekētanga i roto i ngā whakamātautau e pā ana ki te CSF.

Te maimoatanga:

- E rua wiki o te rongoā VLP, 5% te whakahekenga toto ā-taha i te 18 o Hānuere.

- Hanuere 25: Kua puta te 91% o ngā toto ā-taha, i rongoatia ki te CTX, Ara-C, 6-MP chemotherapy.

- 3 o Pēpuere: 22% te pikinga o ngā pahūtanga toto ā-taha.

- 4 o Pēpuere: Kohikohi toto ā-taha autologous 50ml mō te ahurea pūtau CD19-CART.

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

- 13 o Pēpuere (i mua i te werohanga): E whakaatu ana te āhua o te hinu wheua i te 87.5% o ngā pahūtanga, e whakaatu ana te ine rere i te 79.4% o ngā pahūtanga kino.

- Tātaritanga tauanga o te whakakotahitanga ira MLL-ENL: 42.639%.

- 14 o Pēpuere: Te werohanga o ngā pūtau CART i te horopeta o te 5 x 10^5/kg.

- Ngā pānga kino e pā ana ki te CAR-T: Tau 1 CRS (kirika), kāore he paitini roro.

- Te rā 20 i muri i te werohanga: Ka whakaatuhia e te toto i roto i te mimi te tipu o te pukupuku, ko te ōwehenga o ngā pūtau CART he 0.07%.

- Te rongoā pūtau CART koretake.

- 8 Maehe, 2021: Te whakamātautau toto: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.

- Ngā pupuhi toto ā-taha: 83%. Ko te toto ā-taha autologous i kohia he 60ml mō te ahurea pūtau CART takirua CD19/CD22.

- I rongoatia ki te cytarabine me te dexamethasone hei whakahaere i te taumahatanga o te pukupuku.

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

- Maehe 22 (i mua i te werohanga): Te tikanga toto: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. Te āhua o te toto ā-taha: 6% ngā pahūtanga.

- Te āhua o te hinu wheua: 91% ngā pahūtanga. Ngā toenga i roto i te hinu wheua: 88.61% ngā pūtau e whakaatu ana i te CD38, CD19, cCD79a, CD81, CD22, e tohu ana i ngā pūtau ...

- Tātaritanga tauanga o te whakakotahitanga ira MLL-ENL: 62.894%.

- Tātaritanga karyotype ira: 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).

- Maehe 23: Te werohanga o ngā pūtau CART i te horopeta o te 3 x 10^5/kg.

- Maehe 26: Te kirika nui tonu, ā, i muri mai ka puta te pupuhi tinana.

- Maehe 29: Te āhua o te toto ā-taha: 92% ngā pahūtanga; he nui ake ngā transaminases me te bilirubin.

- 2 o Paenga-whāwhā: Te tīmatanga o ngā haurangi, ka rongoatia ki te diazepam.

- Paenga-whāwhā 2 (Rā 10): I tīmata te maimoatanga methylprednisolone mō ngā rā e 3.

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

- 8 o Paenga-whāwhā (Rā 16): E whakaatu ana te aromatawai hinu wheua i te murunga katoa o te āhua, kāore he putanga kino o te pūtau toto; tātaritanga ira whakakotahitanga MLL-ENL: 0.