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

Ngā āhuatanga haumanu:

- Tātaritanga: lymphoma/leukemia lymphoblastic pūtau-T

- Maehe 2020: I puta mai he maremare pākiki me te puranga mediastinal, kua whakaūtia te lymphoma lymphoblastic pūtau-T mā te koiora pokanga papatipu mediastinal.

- I whiwhi ahau i te 8 huringa o te chemotherapy me te neke atu i te 20 wāhanga o te radiotherapy, ā, i tino heke te papatipu o te mediastinum.

- 16 o Hānuere, 2021: I pā mai te mamae ki te waewae matau o raro.

- Te whakamātautau toto: WBC 122.29 x 10^9/L, HGB 91 g/L, PLT 51 x 10^9/L

- Te āhua o te hinu wheua: 95.5% ngā pūtau toto taketake.

- Te ine i te rere o te hinu wheua: 91.77% o ngā pūtau he pūtau-T kāore anō kia pakari.

- Te raupapatanga ira: Kua kitea ngā whakarerekētanga ira i roto i ngā ira NOTCH1, IL7R, ASXL2.

- I whiwhi i te maimoatanga Hyper-CVAD/B, me te maimoatanga ESHAP i muri mai, engari kāore i whai hua ēnei e rua me te kirika tonu.

- 18 o Pēpuere, 2021: I whakaurua ki tō mātou hōhipera.

- I puta mai te kirika, i whakaatuhia e te CT o te uma te niumōnia.

- Te whakamātautau toto: WBC 2.89 x 10^9/L, HGB 57.7 g/L, PLT 14.9 x 10^9/L

- Ngā pūtau toto ā-taha kāore anō kia pakari: 90%

- Te āhua o te hinu wheua: Ngā pūtau nui (taumata IV), 85% ngā pūtau toto taketake.

- Te tātaritanga ā-ira tangata: 87.27% o ngā pūtau he pūtau-T tūturu kino.

- Tātaritanga ira: 46,XX [24]; e toru atu ngā karyotype rerekē i kitea.

- Ngā ira kua whakarerekētia:

1. He pai te whakarerekētanga IL7R T244_I245insARCPL

2. He pai te whakarerekētanga o te NOTCH1 E1583_Q1584dup

3. He pai te whakarerekētanga o te ASXL2 Q602R

- Te whakamātautau ira honohono mate pukupuku toto: Kāore i te pai

- Ngā hua PET/CT: Kāore he kiko pukupuku hypermetabolic nui i roto i te angaanga katoa me te kōhao wheua.

Te maimoatanga:

- I tīmata te rongoā VP chemotherapy, e whai ake nei ngā taipitopito: Vincristine (VDS) 3mg kotahi, Dexamethasone (Dex) 7mg ia 12 hāora mō ngā rā e 9, me te rongoā ārai-mate.

- Maehe 1: Kua heke ngā pūtau toto koretake ki te 7%.

- 4 Maehe: I kohia ngā lymphocytes autologous mō te ahurea pūtau T CD7-CAR.

- 8 Maehe: I tīmata te maimoatanga VLP me te maimoatanga Sida benzamine.

- Maehe 14: I whiwhi i te rongoā rongoā FC (Fludarabine 0.35g mō ngā rā e 3, Cyclophosphamide 45mg mō ngā rā e 3).

- 17 o Poutū-te-rangi (whakaurunga i mua i te pūtau):

- Te tātaritanga ārai mate toenga o te hinu wheua: 15.14% o ngā pūtau e whakaatu ana i te CD7 kanapa, te CD3 mangu, te CD3 cytoplasmic, te delta whakawhāiti i te kaiwhiwhi pūtau T (TCRrd), te whakaaturanga wāhanga o te CD99, e tohu ana i te pūtau T taketake kino.

- Maehe 19: I whakatōkia ngā pūtau T CD7-CAR aunoa (1 x 10^6/kg).

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

- 6 o Paenga-whāwhā (Rā 17): I kitea te māmā o te āhua o te hinu wheua, kāore i kitea e ngā inenga rere o te toto ngā pūtau kino taketake.