Te Mate Pūkahukahu Lymphoblastic Koretake (T-ALL)-02

Ngā Āhuatanga Hauora:

- Tātaritanga: Te mate pukupuku toto pūtau-T whakapeka

- Tīmatanga: Te mutunga o Poutū-te-rangi 2018

- Ngā tohu tuatahi: He nui te rahi o ngā kōpū lymph puta noa i te tinana

- Te whakamātautau toto tuatahi: WBC: 39.46*10^9/L, Hb: 129g/L, PLT: 77*10^9/L

- Te āhua o te hinu wheua: 92% ngā pahūtanga

-Te ine i te rere o te toto: 95.3% ngā pūtau rerekē e whakaatu ana i te

TdT+CD99+CyCD3+CD7stCd5DdimCD4-CD8-mCD3-CD45dim

- Ngā ira honohono: Kino

- Te whakarerekētanga ira: Kua kitea te whakarerekētanga ira NOTCH1

- Tātaritanga ira tangata: Karyotype noa

Hītori Maimoatanga:

- 3 o Paenga-whāwhā, 2018: Whakaora whakaoho me te tikanga VDCP

- 18 Paenga-whāwhā, 2018: Ko te 96% o ngā pūngao wheua

- 20 Paenga-whāwhā, 2018: I whiwhi i te murunga i muri i te maimoatanga CAG

- 18 Mei, 2018: Whakaora whakakotahi me te tikanga CMG+VP

- 22 o Hune, 2018: Kua piki ake ngā pahūtanga o te hinu wheua ki te 40%, kua hoki anō te mate toto toto

- 25 o Hūrae, 2018: Te rongoā CLAM (clarithromycin+cyclophosphamide+amikacin)

- Te whakawhitinga pūtau take hematopoietic mai i te tuakana/teina e ōrite ana ki te HLA mā te whakamahi i te whakatikatika FLU+BU i te 14 o Ākuhata

- Te aroturuki i muri i te whakawhitinga wheua: Te murunga o te āhua o te hinu wheua i te 1 marama, 3 marama, 6 marama, 9 marama, me te 11 marama

- I whakaatuhia te āhua o te hinu wheua kua marino i te 16 marama i muri i te whakawhitinga, ā, i whakaatuhia e te flow cytometry he 0.02% ngā pūtau toto kino kāore anō kia pakari.

- 13 o Noema, 2020: Ko te chimerism toto ā-taha mai i te pūtake kaituku he 97.9%

- Ngā pūtau toto taketake o te taha: 20%

- 18 Hakihea 2020: Te āhua o te hinu wheua: 60.6% ngā pahūtanga

- Te ine i te rere o te toto: 30.85% ngā pūtau toto T kino kāore i te pakari

- Tātaritanga ira: 46, XY (20)

- I whiwhi i te rongoā chemotherapy DA i te 19 o Hānuere, 2021

- Te āhua o te hinu wheua i te 19 o Hānuere, 2021: Te hyperplasia taumata III, 16% ngā pahūtanga

- Tātaritanga karyotype ira: 46, XY (20)

- Te tātaritanga rere: 7.27% o ngā pūtau (i roto i ngā pūtau karihi) i whakaatu i te CD99bri, CD13, CD38, cbcl-2, cCD3, HLA-ABC bri, CD7bri, me te CD5dim i whakaatu hapa, e tohu ana i ngā pūtau toto T kino kāore anō kia pakari.

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

- Tātaritanga whakarerekētanga o te pūtau toto (86 momo):

1. He pai te whakarerekētanga o te PHF6 K299Efs*13

2. He pai te whakarerekētanga o te RUNX1 S322*

3. He pai te whakarerekētanga o te FBXW7 E471G

4. He pai te whakarerekētanga o te JAK3 M511I

5. He pai te whakarerekētanga o te NOTCH1 Q2393*

Te maimoatanga:

- 22 o Hānuere: Te kohikohi me te ahurea o ngā lymphocytes toto ā-roto mō te CD7-CART

- I mua i te werohanga CD7-CART, i whiwhi te tūroro i te VLP (vincristine, l-asparaginase, prednisone) me te chemotherapy bortezomib.

- 3 o Pēpuere: Te rongoā FC mō te chemotherapy (Rewharewha 50mg mō ngā rā e 3 + CTX 0.45g mō ngā rā e 3)

- 5 o Pēpuere (i mua i te werohanga): I whakaatuhia e te āhua o te hinu wheua he 23% ngā pahūtanga.

- I whakaatuhia e te tātaritanga rerenga pūtau he 4.05% ngā pūtau e whakaatu ana i te CD99bri, CD5dim, CD7bri, TDT, cCD3, e tohu ana i ngā pūtau toto T kino kāore anō kia pakari.

- Tātaritanga ira: 46, XY (20)

- Tātaritanga kimera (whai muri i te HSCT): E 52.19% te nui o ngā pūtau i ahu mai i te kaituku.

- 7 o Pēpuere: Te werohanga o ngā pūtau CD7-CART aunoa i te horopeta o te 5*10^5/kg.

- 15 o Pēpuere: Kua heke ngā pūtau toto koretake ki te 2%.

- 19 o Pēpuere (Rā 12 i muri i te werohanga): I pāngia te tūroro e te kirika, ā, e 5 ngā rā i mua i te whakatutukitanga o te whakahaere pāmahana.

- Maehe 2: I whakaatuhia e te aromatawai hinu wheua te murunga katoa o te āhua, ā, kāore i kitea e te flow cytometry ngā pūtau kino kāore anō kia pakari.