Chirwere cheAcute Lymphoblastic Leukemia (T-ALL)-01

Zvinhu zvekurapa:

- Kuongorora: T-cell lymphoblastic lymphoma/leukemia

- Kurume 2020: Akaonekwa nekukosora kweparoxysmal uye mediastinal mass, T-cell lymphoblastic lymphoma yakasimbiswa ne biopsy ye mediastinal mass puncture.

- Ndakarapwa kasere ndichipiwa mishonga yekurapa (chemotherapy) uye ndakarapwa neradiotherapy kanopfuura makumi maviri, zvikaita kuti huwandu hwemediastinal mass huderere zvakanyanya.

- Ndira 16, 2021: Ndakatanga kurwadziwa mugumbo rezasi rerudyi.

- Ropa rinoitwa nguva dzose: WBC 122.29 x 10^9/L, HGB 91 g/L, PLT 51 x 10^9/L

- Maumbirwo emubofu: 95.5% lymphoblasts dzepakutanga.

- Kuyerera kwemubofu muropa: 91.77% yemasero aive maT-cell lymphoblasts asina kukura.

- Kutevedzana kwemajini: Kuchinja kwemajini eNOTCH1, IL7R, ASXL2 kwakaonekwa.

- Ndakawana mushonga weHyper-CVAD/B, mushonga weESAP ukapera, zvese zvikasashanda kana paine fivha isingaperi.

- Kukadzi 18, 2021: Ndakapinda muchipatara chedu.

- Akabatwa nefivha, CT yechipfuva yakaratidza pneumonia.

- Ropa rinoitwa nguva dzose: WBC 2.89 x 10^9/L, HGB 57.7 g/L, PLT 14.9 x 10^9/L

- Masero asina kukura eropa ari kumucheto: 90%

- Maumbirwo emapfupa emubofu: Hypercellular (IV grade), 85% primitive lymphoblasts.

- Kugadzira immunophenotyping: 87.27% yemasero aive ma lymphoblast eT-cell ekutanga ane malignant.

- Kuongororwa kwechromosome: 46,XX [24]; mamwe ma karyotype matatu asina kujairika akaonekwa.

- Majini akachinja:

1. IL7R T244_I245insARCPL mutation ine positive

2. NOTCH1 E1583_Q1584dup mutation ine positive

3. ASXL2 Q602R shanduko ine hutachiona

- Kuongororwa kwemajini eLeukemia fusion: Negative

- Mhedzisiro yePET/CT: Hapana bundu rakawanda rinokonzera metabolic bundu mumapfupa ese nemapfupa.

Kurapa:

- Kutanga kurapwa kweVP chemotherapy, kwakatsanangurwa sezvinotevera: Vincristine (VDS) 3mg kamwe chete, Dexamethasone (Dex) 7mg maawa gumi nemaviri ega ega kwemazuva mapfumbamwe, pamwe chete nekurapwa kwekudzivirira hutachiona.

- Kurume 1: Masero eropa asina kukura akaderera kusvika pa7%.

- Kurume 4: Ma lymphocytes e autologous akaunganidzwa kuti akwanise kukura kwemasero eCD7-CAR T.

- Kurume 8: Takatanga chirongwa cheVLP pamwe chete nekurapwa neSida benzamine.

- Kurume 14: Ndakapiwa mushonga weFC regimen chemotherapy (Fludarabine 0.35g kwemazuva matatu, Cyclophosphamide 45mg kwemazuva matatu).

- Kurume 17 (kuiswa kwemasero asati aiswa):

- Kuchengetedza immunophenotyping yemapfupa: 15.14% yemasero anoratidza kupenya kweCD7, CD3 dim, cytoplasmic CD3, T cell receptor restricted delta (TCRrd), kuratidzwa kweCD99 zvishoma, zvichiratidza masero eT anouraya.

- Kurume 19: Masero eCD7-CAR T akapinzwa muropa (1 x 10^6/kg).

- Migumisiro ine chekuita neCAR-T: CRS yeGiredhi 1 (fivha), hapana chepfu muuropi.

- Kubvumbi 6 (Zuva re17): Maumbirwo emapfupa emubofu akaratidza kurerukirwa, flow cytometry haina kuona masero ekare ane malignant.