Acute Lymphoblastic Leukemia(T-ALL)-01

Vaega fa'apitoa:

- Su'esu'ega: T-cell lymphoblastic lymphoma/leukemia

- Mati 2020: Tu'u atu ile tale paroxysmal ma mediastinal mass, fa'amaonia le T-cell lymphoblastic lymphoma e ala ile biopsy tu'i tu'i tele.

- Na maua le 8 taamilosaga o le chemotherapy ma sili atu i le 20 vasega o le leitio, e mafua ai le faʻaitiitia o le tele o mediastinal.

- Ianuari 16, 2021: Atia'e le tiga i le itu taumatau pito i lalo.

- Fa'asologa o le toto: WBC 122.29 x 10^9/L, HGB 91 g/L, PLT 51 x 10^9/L

- Su'i ponaivi morphology: 95.5% lymphoblasts muamua.

- Cytometry o le tafe o ga'o ponaivi: 91.77% o sela o lymphoblasts T-cell e le'i matutua.

- Fa'asologa o kenera: O suiga i kenera NOTCH1, IL7R, ASXL2 ua iloa.

- Maua le Hyper-CVAD/B regimen, ESHAP regimen mulimuli ane, e le aoga uma i le fiva faifai pea.

- Fepuari 18, 2021: Faʻatagaina i le matou falemaʻi.

- Na maua i le fiva, o le fatafata CT na iloa ai le niumonia.

- Fa'asologa o le toto: WBC 2.89 x 10^9/L, HGB 57.7 g/L, PLT 14.9 x 10^9/L

- O le toto pito pito e le'i matutua sela: 90%

- Su'i ponaivi: Hypercellular (vaega IV), 85% lymphoblasts muamua.

- Immunophenotyping: 87.27% o sela o ni lymphoblasts T-cell tuai.

- Su'esu'ega Chromosomal: 46,XX [24]; e tolu isi karyotypes fa'aletonu na matauina.

- Genes ua suia:

1. IL7R T244_I245insARCPL suiga lelei

2. NOTCH1 E1583_Q1584dup mutation lelei

3. ASXL2 Q602R suiga lelei

- Su'esu'ega o kenera fusu'ai le leukemia: Le lelei

- PET/CT fa'ai'uga: Leai se fa'aautu tele tumo hypermetabolic i le auivi atoa ma le ga'o o ponaivi.

Togafitiga:

- Amata le VP regimen chemotherapy, fa'amatalaina e pei ona taua i lalo: Vincristine (VDS) 3mg tasi, Dexamethasone (Dex) 7mg i 12 itula mo le 9 aso, fa'atasi ai ma togafitiga fa'ama'i.

- Mati 1: Fa'aiti'itia sela e le'i matutua i totonu o le toto i le 7%.

- Mati 4: Aoina mai le autologous lymphocytes mo CD7-CAR T cell culture.

- Mati 8: Amata VLP faiga fa'atasi ma Sida benzamine togafitiga.

- Mati 14: Maua FC regimen chemotherapy (Fludarabine 0.35g mo 3 aso, Cyclophosphamide 45mg mo 3 aso).

- Mati 17 (a'o le'i fa'asu'ai sela):

- Immunophenotyping ole ga'o ponaivi: 15.14% sela e fa'aalia le CD7 malamalama, CD3 fa'apouliuli, cytoplasmic CD3, T cell receptor fa'atapula'aina delta (TCRrd), vaega fa'aaliga o le CD99, e fa'aalia ai le leaga o sela T muamua.

- Mati 19: Fa'auluina autologous CD7-CAR T sela (1 x 10^6/kg).

- CAR-T a'afiaga: Vasega 1 CRS (fiva), leai se neurotoxicity.

- Aperila 6 (Aso 17): O le su'esu'e o ga'o o ponaivi na fa'aalia ai le fa'amagaloga, o le flow cytometry e le'i iloa ai ni sela fa'ama'i.