Acute Lymphoblastic Leukemia(B-ALL)-01

Onosa'i: Tagata XX

Itupa：Tane

Tausaga: 24 tausaga

Nationality: Saina

Su'esu'ega: Acute Lymphoblastic Leukemia(B-ALL)

Faafesootai matou

Na maua i le ma'i B-cell lymphoblastic leukemia ia Novema 28, 2017.

Togafitiga ile faiga ole VDLP ile taimi muamua, e maua ai se fa'amagaloga ole ga'o ponaivi (fa'amatalaga e le'i lipotia).

Fepuari 2018: Suia ile VLCAM regimen. O su'esu'ega o le tafe o ga'o o ponaivi na fa'aalia ai le 60.13% o sela B e matua leaga.

Mati 2018: Fa'aulu ile BiTE fa'ata'ita'iga falema'i. Fa'amagaloga fa'aletino ile ga'o ponaivi, leai ni sela fa'atama'i leaga e maua ile cytometry.

Me 8, 2018: Maua TBI/CY+VP16 conditioning regimen sosoo ai ma allogeneic stem cell transplant from fully matched sibling (AB+ donor to A+ recipient). Neutrophil toe faʻaleleia i le aso +11, toe faʻaleleia megakaryocyte i le aso +12.

Tesema 5, 2018: Fa'amuta atoa i le ga'o ponaivi, leai ni sela fa'atama'i leaga e iloa e le cytometry o le tafe. Na maua le foaʻi lymphocyte infusion (DLI) ma togafitiga faʻamalosi ma le dasatinib ma le imatinib e puipuia ai le toe faʻafoʻi.

Fepuari 2, 2019: Morphology na faʻaalia ai le 6.5% o sela e le o matua, o le cytometry o le tafe na faʻaalia ai le 0.08% o lymphoblasts B e le o matua. Na maua togafitiga DLI. Mati 28, 2019: Flow cytometry na faʻaalia e leai se faʻalavelave.

Aokuso 11, 2019: Toe fo'i le ga'o ponaivi, togafitia i le dasatinib.

Setema 2, 2019: Morphology na faʻaalia ai le 3% o sela e le o matua, o le cytometry o le tafe na faʻaalia ai le 0.04% o sela e le matutua. Faʻaauau togafitiga faʻatasi ma le dasatinib, sosoo ai ma le 2 taamilosaga o le chemotherapy methotrexate.

Me 11, 2020: Toe fo'i le ga'o ponaivi.

Na maua 2 autologous CD19-CAR-T cell therapy ma 2 allogeneic CD19-CAR-T cell therapy i le 2020, e leai se tasi na maua le fa'amagaloga.

Oketopa 26, 2020: Faʻatagaina i le matou falemaʻi.

Su'esu'ega a le Falesu'esu'e:

Fa'asologa o le toto: WBC 22.75 x 10^9/L, HGB 132 g/L, PLT 36 x 10^9/L

toto pito pito e le'i matutua sela: 63%

Fa'ailoga o ga'o o ponaivi: Hypercellular (vasega II), 96% lymphoblasts e le'i matutua.

Immunophenotyping: O sela e faʻaalia CD19, cCD79a, CD38dim, CD10bri, CD34, CD81dim, CD24, HLA-DR, TDT, CD22, CD72; fa'aaliga vaega ole CD123. Fa'ailoaina o ni lymphoblast B e matua'i leaga.

Suiga ole tuma toto: Leaga.

Leukemia fusu'aga kene: NUP214-ABL1 fusi fa'aigoa lelei.

Iloiloga o Chromosome: 46, XX, t(1;9)(p34;p24), faaopoopo(11)(q23)[4]/46, XX, t(1;9)(p34;p24), faaopoopo(11) (q23)x2 [2]/46, XX[3]

Chimerism: O sela e maua mai i le Donor e maua ai le 7.71%.

Togafitiga:

- VDS, DEX, LASP fa'ata'ita'iga o le chemotherapy.

- Novema 20: Selula e le o matua i le toto peripheral 0%.

- Aoina o lymphocytes toto peripheral autologous mo CD19/22 lua CAR-T aganuu cell.

- Novema 29: FC regimen chemotherapy (Flu 50mg x 3, CTX 0.4gx 3).

- Tesema 2 (a'o le'i tu'uina le CAR-T cell infusion):

- Fa'asologa o le toto: WBC 0.44 x 10^9/L, HGB 66 g/L, PLT 33 x 10^9/L.

- Su'i ponaivi: Hypercellular (vasega IV), 68% lymphoblasts le matutua.

- Su'esu'ega fa'atele ole ga'o fusifusia NUP214-ABL1: 24.542%.

- Flow cytometry: 46.31% o sela faʻaalia CD38dim, CD22, BCL-2, CD19, CD10bri, CD34, CD81dim, CD24, cCD79a, e faʻaalia ai le leaga B lymphoblasts e le matutua.

- Tesema 4: Infusion o autologous CD19/22 lua CAR-T sela (3 x 10^5/kg).

- A'afiaga o le CAR-T: Vasega 1 CRS, fiva ile Aso 6 ma le Tmax ole 40°C, fiva e pulea ile Aso 10. Leai se neurotoxicity na matauina.

- Tesema 22 (Aso 18 su'esu'ega): Fa'amagaloga atoatoa i le ga'o ponaivi, leai ni sela fa'atama'i leaga e iloa ile cytometry. Su'esu'ega fa'atele ole gene fusifusia NUP214-ABL1: 0%.