Ọrịa kansa ọbara Lymphoblastic dị oke njọ (T-ALL)-02

Atụmatụ Ahụike:

- Nchọpụta: Ọrịa leukemia lymphoblastic T-cell dị egwu

- Mmalite: Ngwụcha Maachị 2018

- Mgbaàmà mbụ: Ọtụtụ mmụba nke lymph node elu n'ahụ dum

- Usoro ọbara mbụ: WBC: 39.46*10^9/L, Hb: 129g/L, PLT: 77*10^9/L

- Ọdịdị nke ụmị ọkpụkpụ: 92% mgbawa

- Sistemụ mmiri na-agba: 95.3% mkpụrụ ndụ na-adịghị mma na-egosipụta

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

- Mkpụrụ ndụ ihe nketa njikọta: Ihe na-adịghị mma

- Mmụgharị mkpụrụ ndụ ihe nketa: Achọpụtara mmụgharị mkpụrụ ndụ ihe nketa NOTCH1

- Nyocha Chromosome: Karyotype nkịtị

Akụkọ Ọgwụgwọ:

- Eprel 3, 2018: Ọgwụgwọ induction site na usoro VDCP

- Eprel 18, 2018: Mgbọ ọkpụkpụ nwere 96%

- Eprel 20, 2018: E nwetara mgbaghara mgbe usoro CAG gasịrị

- Mee 18, 2018: Ọgwụgwọ njikọta na usoro CMG+VP

- Juun 22, 2018: Mwakpo ọkpụkpụ amụbaala ruo 40%, ọrịa leukemia na-alaghachi azụ

- Julaị 25, 2018: Usoro CLAM (clarithromycin+cyclophosphamide+amikacin)

- Ịmịnye mkpụrụ ndụ hematopoietic site na nwanne HLA nke dabara na ya site na iji FLU+BU conditioning na Ọgọst 14

- Nlekota mgbe a gbanyechara ya: Mwepụ nke ọdịdị ọkpụkpụ mgbe ọnwa 1, ọnwa 3, ọnwa 6, ọnwa 9, na ọnwa 11 gasịrị

- Ọdịdị nke ụmị ọkpụkpụ gosipụtara mgbaghara mgbe ọnwa iri na isii gachara ịwa ahụ, ebe cytometry na-apụta na lymphocytes na-etobeghị nke ọma 0.02%.

- Nọvemba 13, 2020: Mmegharị ọbara nke dị n'akụkụ site n'aka onye nyere onyinye bụ 97.9%

- Mkpụrụ ndụ mbụ nke ọbara dị n'akụkụ: 20%

- Disemba 18, 2020: Ọdịdị ọkpụkpụ: 60.6% mgbawa

- Cytometry mmiri: 30.85% malignant malignant T lymphocytes

- Nyocha nke kromosome: 46, XY (20)

- Enyere m ọgwụgwọ DA regimen chemotherapy na Jenụwarị 19, 2021

- Ọdịdị ọkpụkpụ ọkpụkpụ na Jenụwarị 19, 2021: Hyperplasia nke ọkwa nke atọ, 16% mgbawa

- Nyocha karyotype chromosome: 46, XY (20)

- Cytometry mmiri: 7.27% nke mkpụrụ ndụ (n'etiti mkpụrụ ndụ nuklia) e gosipụtara CD99bri, CD13, CD38, cbcl-2, cCD3, HLA-ABC bri, CD7bri, na CD5dim nke e gosipụtara n'akụkụ, na-egosi lymphocytes T na-etobeghị eto nke ọma

- Nnyocha mkpụrụ ndụ ihe nketa nke leukemia: Ihe na-adịghị mma

- Nnyocha mgbanwe mkpụrụ ndụ ọbara (ụdị 86):

1. Mmụba PHF6 K299Efs*13 dị mma

2. Mmụba RUNX1 S322* dị mma

3. Mmụba FBXW7 E471G dị mma

4. Mmụba JAK3 M511I dị mma

5. NOTCH1 Q2393* mgbanwe dị mma

Ọgwụgwọ:

- Jenụwarị 22: Nchịkọta na mmepe nke sel ọbara lymphocytes maka CD7-CART

Tupu e nye onye ọrịa ọgwụ CD7-CART, e nyere ya ọgwụ VLP (vincristine, l-asparaginase, prednisone) tinyere ọgwụ bortezomib chemotherapy.

- Febụwarị 3: Usoro ọgwụgwọ FC (Flu 50mg maka ụbọchị 3 + CTX 0.45g maka ụbọchị 3)

- Febụwarị 5 (tupu a gbanye ya): Ọdịdị ọkpụkpụ gosiri na ọ gbawara 23%.

- Cytometry nke mmiri na-agbapụta gosiri 4.05% mkpụrụ ndụ na-egosipụta CD99bri, CD5dim, CD7bri, TDT, cCD3, nke na-egosi mkpụrụ ndụ T ndị na-adịghị ike.

- Nyocha nke kromosome: 46, XY (20)

- Nyocha nke chimerism (mgbe HSCT gasịrị): Mkpụrụ ndụ sitere na ndị nyere onyinye ruru 52.19%.

- Febụwarị 7: Ntinye mkpụrụ ndụ CD7-CART onwe ya na dose nke 5*10^5/kg.

- Febụwarị 15: Mkpụrụ ndụ ọbara na-etobeghị eto belatara ruo 2%.

- Febụwarị 19 (Ụbọchị nke 12 mgbe a mịnyechara ya): Onye ọrịa ahụ malitere ịrịa ahụ ọkụ, nke dịgidere ruo ụbọchị ise tupu e nwee njikwa okpomọkụ.

- Machị 2: Nnyocha ụmị ọkpụkpụ gosiri na e wepụrụ morphological kpamkpam, ebe cytometry na-apụta n'ime ahụ anaghị achọpụta mkpụrụ ndụ ndị na-etobeghị nke ọma.