Khansa ya m'magazi ya Lymphoblastic (T-ALL)-02

Zinthu Zachipatala:

- Kuzindikira: Matenda a khansa ya m'magazi ya T-cell lymphoblastic

- Chiyambi: Kumapeto kwa Marichi 2018

Zizindikiro zoyamba: Kutupa kwa ma lymph node ambiri m'thupi lonse

- Kuyamba kwa ndondomeko ya magazi: WBC: 39.46*10^9/L, Hb: 129g/L, PLT: 77*10^9/L

- Kapangidwe ka mafupa: 92% kuphulika

-Flow cytometry: 95.3% ya maselo osazolowereka omwe amatulutsa

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

- Majini osakanikirana: Oipa

- Kusintha kwa majini: Kusintha kwa majini a NOTCH1 kwapezeka

- Kusanthula kwa chromosome: Karyotype yachibadwa

Mbiri ya Chithandizo:

- Epulo 3, 2018: Chithandizo choyambitsa matenda pogwiritsa ntchito njira ya VDCP

- Epulo 18, 2018: Kuphulika kwa mafupa kunali 96%

- Epulo 20, 2018: Ndapeza mpumulo pambuyo pa ndondomeko ya CAG

- Meyi 18, 2018: Kuphatikiza mankhwala ndi CMG+VP regimen

- Juni 22, 2018: Kuphulika kwa mafupa kunakwera kufika pa 40%, kubwereranso kwa khansa ya m'magazi

- Julayi 25, 2018: Mankhwala a CLAM (clarithromycin+cyclophosphamide+amikacin)

- Kusamutsa maselo oyambira a magazi kuchokera kwa m'bale wa HLA-matched pogwiritsa ntchito FLU+BU conditioner pa Ogasiti 14

- Kuyang'anira momwe mafupa amaonekera akangobereka mwana: Kutuluka kwa mafupa m'thupi pakatha mwezi umodzi, miyezi itatu, miyezi isanu ndi umodzi, miyezi 9, ndi miyezi khumi ndi umodzi.

- Kapangidwe ka mafupa a m'mafupa kanasonyeza kuti maselo a m'mafupa asiya kugwira ntchito patatha miyezi 16 atangobereka, ndipo njira yoyendera magazi inasonyeza kuti maselo a m'mafupa omwe anali ndi vuto lalikulu anali 0.02% mwa maselo a m'mafupa omwe anali ndi vuto lalikulu.

- Novembala 13, 2020: Kuchuluka kwa magazi m'magazi kuchokera kwa omwe adapereka magazi kunali 97.9%

- Maselo oyambira a magazi ozungulira: 20%

- Disembala 18, 2020: Kapangidwe ka mafupa: 60.6% kuphulika

- Flow cytometry: 30.85% malignant immature T lymphocytes

- Kusanthula kwa chromosome: 46, XY (20)

- Analandira chithandizo cha DA cha mankhwala a chemotherapy pa Januware 19, 2021

- Kapangidwe ka mafupa pa Januware 19, 2021: Hyperplasia ya Giredi III, 16% ya kuphulika

- Kusanthula kwa karyotype ya chromosome: 46, XY (20)

- Flow cytometry: 7.27% ya maselo (pakati pa maselo a nyukiliya) omwe ali ndi CD99bri, CD13, CD38, cbcl-2, cCD3, HLA-ABC bri, CD7bri, ndi CD5dim yomwe ili ndi kachilombo pang'ono, zomwe zikusonyeza kuti ma lymphocyte a T osakhwima ndi owopsa.

- Kuwunika majini osakanikirana ndi khansa ya m'magazi: Zoyipa

- Kusanthula kusintha kwa khansa ya m'magazi (mitundu 86):

1. PHF6 K299Efs*13 kusintha kwa thupi

2. RUNX1 S322* yapezeka ndi kusintha kwa majini

3. FBXW7 E471G yapezeka ndi kusintha kwa majini

4. JAK3 M511I yasintha kukhala ndi kachilombo ka HIV

5. NOTCH1 Q2393* kusintha kwa thupi

Chithandizo:

- Januwale 22: Kusonkhanitsa ndi kupanga ma lymphocyte a magazi a autologous peripheral kuti apeze CD7-CART

- Asanalandire chithandizo cha CD7-CART, wodwalayo adalandira VLP (vincristine, l-asparaginase, prednisone) komanso bortezomib chemotherapy.

- February 3: Chithandizo cha mankhwala a FC regimen (Flu 50mg kwa masiku atatu + CTX 0.45g kwa masiku atatu)

- February 5 (asanalowe m'thupi): Kapangidwe ka mafupa a m'mafupa kanasonyeza kuphulika kwa 23%.

- Flow cytometry inapeza maselo 4.05% omwe ali ndi CD99bri, CD5dim, CD7bri, TDT, cCD3, zomwe zikusonyeza ma lymphocyte a T osakhwima omwe ali ndi khansa.

- Kusanthula kwa chromosome: 46, XY (20)

- Kusanthula kwa Chimerism (pambuyo pa HSCT): Maselo otengedwa ndi opereka anali 52.19%.

- February 7: Kulowetsedwa kwa maselo a autologous CD7-CART pa mlingo wa 5*10^5/kg.

- February 15: Maselo osakhwima m'magazi a m'mphepete mwa nyanja atsika kufika pa 2%.

- February 19 (Tsiku la 12 atalandira mankhwala): Wodwalayo anayamba kutentha thupi, komwe kunatenga masiku 5 kutentha kusanathe.

- Marichi 2: Kuwunika kwa mafupa kunawonetsa kuchira kwathunthu kwa morphological, ndipo flow cytometry sinazindikire maselo osakhwima omwe ali ndi khansa.