Acute Lymphoblastic Leukemia(B-ALL)-02
Itu uiga:
- Me 19, 2019: Ua maua i le ma'i B-cell lymphoblastic leukemia (B-ALL)
- Faʻaalia i le tele o paʻu o le ulu ma le lymphadenopathy
- Fa'asologa o le toto: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, lymphocytes fa'aletonu 4%
- Va'aiga o ga'o o ponaivi: 80.2% lymphoblasts e le'i matutua (pa'i)
- Immunophenotyping: 74.19% o sela e leaga B-laina muamua sela o loʻo faʻaalia CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, faʻaalia vaega cIgM. Su'esu'ega: B-UMA (Laasaga a'o le'i-B)
- Fusion kene: MLL-ENL lelei, Philadelphia chromosome-pei (Ph-pei) mata leaga
- Chromosome: 46, XX, t(11;19)(q23;p13), del(20)(q12) [3]/46, XX [7]
- VDLD regimen chemotherapy na maua muamua le fa'amagaloga fa'ama'i ina ua uma le 1 masina, MLL-ENL quantitative PCR 0.026%
- Fa'aauau le chemotherapy e tusa ai ma tulafono a tamaiti, MLL-ENL quantitative PCR 0 pe a uma le taamilosaga lona 4. Sa faaauau pea le isi chemotherapy.
- Mati 2020: Fa'ama'i fa'ama'i o totoe ole ga'o ole ponaivi 0.35%, MLL-ENL quantitative PCR 0.53%, e fa'ailoa mai ai le aga'i i tua. Na teena e le aiga le totoina. Fa'aauau le chemotherapy mo le 3 taamilosaga.
- Iulai 2020: Ua toe solomuli le ga'o ponaivi.
- Novema 11, 2020: Intrathecal chemotherapy, CSF immunological residual disease 66%, ua maua i le leukemia o le tino tutotonu. Fa'alua fa'alua le togafitiga fa'a-intrathecal, fa'aletonu le CSF.
- Tesema 31, 2020: Faʻatagaina i le matou falemaʻi.
- Fa'asologa o le toto: WBC 3.99 x 10^9/L, HGB 66 g/L, PLT 57 x 10^9/L
- Faitauga o le sasaina o le toto: 69%
- Va'aiga o ga'o o ponaivi: 90% lymphoblasts e le'i matutua (pa'i)
- Immunophenotyping: 84.07% o sela e faʻaalia le CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, faʻaalia vaega CD24, CD15dim, faʻaalia le leaga B lymphoblasts e le matutua.
- Fusion gene: MLL-ENL fusion gene lelei, quantitative PCR 44.419%
- Suiga o le kenera: KMT2D suiga lelei (germline tupuaga)
- Chromosome karyotype: 46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3), add( 14)(q34), -17, +mar [7]/46, idem, t(3;16)(p21;p13.3) [1]/46, XX [13]
- PET-CT: Faʻateleina le faʻatupuina o le gasegase i totonu o le skeletal ma le gaʻo ponaivi atoa, maualuga masalosaloga o le leukemia toe tupu; splenomegaly faatasi ai ma le faateleina o le metabolism, atonu e aafia ai le leukemia.
- Fa'atino tu'i le lumbar ma le chemotherapy fa'atasi, leai ni fa'aletonu e maua ile su'ega ole CSF.
Togafitiga:
- Lua vaiaso ole togafitiga ole VLP, e 5% ole toto ile peripheral ile aso 18 Ianuari.
- Ianuari 25: E 91% le sasaina o le toto toto, togafitia ile CTX, Ara-C, 6-MP chemotherapy.
- Fepuari 3: Fa'asao toto toto 22%.
- Fepuari 4: Aoina mai le 50ml autologous peripheral toto mo CD19-CART cell culture.
- MTX 1g, FC chemotherapy (Flu 15mg i aso uma x 3 aso, CTX 0.12g aso uma x 3 aso).
- Fepuari 13 (a'o le'i fa'ainu): E 87.5% le pa'u o le ga'o o ponaivi, fa'apea fo'i le 79.4%.
- MLL-ENL fusion gene su'esu'ega fa'atusatusaga: 42.639%.
- Fepuari 14: Fa'asalaina sela CART ile fua ole 5 x 10^5/kg.
- A'afiaga leaga e fa'atatau i le CAR-T: Vasega 1 CRS (fiva), leai se neurotoxicity.
- Aso 20 ina ua uma ona tui: O le su'eina o le toto o lo'o fa'aalia ai le fa'atuputeleina o le tuma, CART vaega ole sela 0.07%.
- Le aoga ole CART cell therapy.
- Mati 8, 2021: Fa'asologa o le toto: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.
- Fa'asao toto pito i fafo: 83%. Autologous toto peripheral aoina 60ml mo CD19/CD22 lua CART aganuu cell.
- Togafiti ile cytarabine ma le dexamethasone e pulea ai le mamafa ole tuma.
- Mati 18: FC chemotherapy (Flu 15mg i aso uma x 3 aso, CTX 0.12g aso uma x 3 aso).
- Mati 22 (a'o le'i fa'asusu): Fa'asologa o le toto: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. Fa'ata'otoga o le toto pito i tua: 6% pa'u.
- Su'i ponaivi: 91% fa'alavelave. O totoe i le ga'o ponaivi: 88.61% sela o lo'o fa'aalia le CD38, CD19, cCD79a, CD81, CD22, e fa'aalia ai ni lymphoblasts B e le'i matutua.
- MLL-ENL fusion gene su'esu'ega aofa'i: 62.894%.
- Chromosome karyotype su'esu'ega: 46, XX, del(1)(p36.1), del(11)(q13), t(11;19)(q23;p13.3), add(14)(q34), - 17, +mar [2]/46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3 ), faaopoopo(14)(q34).
- Mati 23: Fa'asalaina sela CART ile fua ole 3 x 10^5/kg.
- Mati 26 aga'i i luma: Fa'aauau le fiva maualuga ma fa'atupuina ai le edema fa'apitoa.
- Mati 29: Fa'ata'ita'iga o le toto pito i luga ole toto: 92% fa'alavelave; maualuga transaminases ma bilirubin.
- Aperila 2: Amata o faoa faamalosi, togafitia i le diazepam.
- Aperila 2 (Aso 10): Amata togafitiga methylprednisolone mo 3 aso.
- Tali a le CRS: Vasega 3, CRES: Vasega 3.
- Aperila 8 (Aso 16): O su'esu'ega o ga'o o ponaivi e fa'aalia ai le fa'amagaloina atoatoa o le tino, fa'aletonu le tafe o le cytometry mo tu'i leaga; MLL-ENL fusion gene su'esu'ega aofa'i: 0.
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