ʻO ka maʻi Leukemia Lymphoblastic ʻoʻoleʻa (T-ALL)-02

Nā Hiʻohiʻona Lapaʻau:

- Hōʻike: Leukemia lymphoblastic T-cell koʻikoʻi

- Hoʻomaka: Hopena o Malaki 2018

- Nā hōʻailona mua: Hoʻonui nui ʻia nā lymph node superficial ma ke kino holoʻokoʻa

- Ka papa hana koko mua: WBC: 39.46*10^9/L, Hb: 129g/L, PLT: 77*10^9/L

- ʻAno o ka iwi ʻiʻo: 92% pahū

-Cytometry kahe: 95.3% nā pūnaewele ʻē aʻe e hōʻike ana

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

- Nā genes Fusion: Maikaʻi ʻole

- Hoʻololi ʻana o ka gene: Ua ʻike ʻia ka hoʻololi ʻana o ka gene NOTCH1

- Ka nānā ʻana i ka chromosome: karyotype maʻamau

Ka Moʻolelo Lapaʻau:

- ʻApelila 3, 2018: Hoʻōla hoʻokomo me ka regimen VDCP

- ʻApelila 18, 2018: ʻO 96% ka nui o nā pahū iwi

- ʻApelila 20, 2018: Ua hoʻokō ʻia ke kala ʻana ma hope o ka regimen CAG

- Mei 18, 2018: Hoʻohui lāʻau lapaʻau me ka regimen CMG+VP

- Iune 22, 2018: Ua piʻi aʻe ka pahū ʻana o ka iwi i 40%, ka hoʻi hou ʻana o ka leukemia

- Iulai 25, 2018: Papa hana CLAM (clarithromycin+cyclophosphamide+amikacin)

- Ke hoʻoili ʻana o nā cell kumu hematopoietic mai ke kaikaina HLA-matched me ka hoʻohana ʻana i ka hoʻomaʻamaʻa FLU+BU ma ʻAukake 14

- Ke nānā ʻana ma hope o ke kāpili ʻana: Ke kala ʻia ʻana o ke ʻano o ka iwi ma 1 mahina, 3 mahina, 6 mahina, 9 mahina, a me 11 mahina

- Ua hōʻike ʻia ke ʻano o ka iwi iwi i ka hoʻomaha ʻana i 16 mau mahina ma hope o ke kāpili ʻana, me ka hōʻike ʻana o ka cytometry kahe i ka 0.02% mau lymphocytes ʻōpiopio malignant

- Nowemapa 13, 2020: ʻO ke kimera koko peripheral mai ke kumu hāʻawi he 97.9%

- Nā pūnaewele koko peripheral primitive: 20%

- Kekemapa 18, 2020: ʻAno o ka iwi ʻōʻō: 60.6% pahū

- Ka cytometry kahe: 30.85% nā lymphocytes T ʻōpiopio ʻino

- Ka nānā ʻana i nā chromosome: 46, XY (20)

- Ua loaʻa iaʻu ka chemotherapy regimen DA ma Ianuali 19, 2021

- ʻAno o ka iwi ʻiʻo ma Ianuali 19, 2021: Hyperplasia Papa III, 16% pahū

- Ka nānā ʻana i ke karyotype chromosome: 46, XY (20)

- Ka cytometry kahe: 7.27% o nā cell (ma waena o nā cell nuklea) i hōʻike i ka CD99bri, CD13, CD38, cbcl-2, cCD3, HLA-ABC bri, CD7bri, a me ka CD5dim i hōʻike hapa ʻia, e hōʻike ana i nā lymphocytes T ʻōpiopio malignant

- Nānā ʻana i ka hui ʻana o ka leukemia: Maikaʻi ʻole

- Ka nānā ʻana i ka hoʻololi ʻana o ka puʻupuʻu koko (86 ʻano):

1. PHF6 K299Efs*13 mutation maikaʻi

2. Loaʻa ka hoʻololi ʻana o RUNX1 S322* maikaʻi

3. Loaʻa ka loli maikaʻi o FBXW7 E471G

4. Maikaʻi ka hoʻololi ʻana o JAK3 M511I

5. NOTCH1 Q2393* mutation maikaʻi

Lapaʻau:

- Ianuali 22: Hōʻiliʻili a me ka moʻomeheu o nā lymphocytes koko peripheral autologous no CD7-CART

- Ma mua o ka hoʻokomo ʻana o CD7-CART, ua loaʻa i ka mea maʻi ka VLP (vincristine, l-asparaginase, prednisone) me ka chemotherapy bortezomib.

- Pepeluali 3: Kemokalama FC regimen (Flu 50mg no 3 lā + CTX 0.45g no 3 lā)

- Pepeluali 5 (ma mua o ka hoʻokomo ʻana): Ua hōʻike ke ʻano o ka iwi iwi i 23% mau pahū.

- Ua hōʻike ka cytometry kahe i ka 4.05% o nā pūnaewele e hōʻike ana i ka CD99bri, CD5dim, CD7bri, TDT, cCD3, e hōʻike ana i nā lymphocytes T ʻōpiopio malignant.

- Ka nānā ʻana i nā chromosome: 46, XY (20)

- Ka nānā ʻana o ka chimerism (ma hope o HSCT): Ua helu ʻia nā pūnaewele i loaʻa mai ka mea hāʻawi no 52.19%.

- Pepeluali 7: Hoʻokomo ʻia ʻana o nā pūnaewele CD7-CART autologous ma kahi mahele o 5 * 10 ^ 5 / kg.

- Pepeluali 15: Ua hoʻemi ʻia nā hunaola koko ʻōpiopio i 2%.

- Pepeluali 19 (Lā 12 ma hope o ke kāwili ʻana): Ua loaʻa i ka mea maʻi ke kuni, a ua mau ia no 5 mau lā ma mua o ka hoʻokō ʻia ʻana o ke kāohi ʻana i ka mahana.

- Malaki 2: Ua hōʻike ka loiloi ʻana o ka iwi iwi i ke kala piha ʻana, me ka ʻike ʻole ʻana o ka cytometry kahe i nā pūnaewele ʻōpiopio ʻino.