Aisan lukimia Lymphoblastic nla (B-ALL) -02

Awọn abuda ọran:

- Oṣu Karun ọjọ 19, Ọdun 2019: Ti ṣe ayẹwo pẹlu aisan lukimia lymphoblastic B-cell nla (B-ALL)

- Agbekalẹ pẹlu ọpọ ọpọ eniyan scalp ati lymphadenopathy

- Ilana ẹjẹ: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, awọn lymphocytes ajeji 4%

- Mofoloji ọra inu egungun: 80.2% awọn lymphoblasts ti ko dagba (awọn aruwo)

- Immunophenotyping: 74.19% ti awọn sẹẹli jẹ awọn sẹẹli iṣaaju B-ila ti o buruju ti n ṣalaye CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, ti n ṣalaye ni apakan cIgM. Ayẹwo: B-ALL (Ipele-tẹlẹ B)

- Jiini Fusion: MLL-ENL rere, Philadelphia chromosome-like (Ph-like) odi iboju

- Chromosome: 46, XX, t (11;19) (q23; p13), del (20) (q12) [3]/46, XX [7]

- Kimoterapi ilana VDLD ni ibẹrẹ idariji ajẹsara lẹhin oṣu kan, MLL-ENL pipo PCR 0.026%

- Kimoterapi tẹsiwaju gẹgẹbi ilana ilana paediatric, MLL-ENL pipo PCR 0 lẹhin ọmọ kẹrin. Kimoterapi siwaju sii tẹsiwaju.

- Oṣu Kẹta 2020: Arun ajẹsara ajẹsara ọra inu egungun 0.35%, MLL-ENL pipo PCR 0.53%, n tọka aṣa si ipadasẹhin. Idile kọ asopo. Kimoterapi tẹsiwaju fun awọn akoko 3.

- Oṣu Keje 2020: Ọra inu egungun tun pada ni kikun.

- Oṣu kọkanla ọjọ 11, Ọdun 2020: chemotherapy intrathecal, arun ajẹsara ajesara CSF 66%, ṣe ayẹwo pẹlu aisan lukimia ti aarin aifọkanbalẹ. Intrathecal chemotherapy tun lemeji, CSF yipada odi.

- Oṣu kejila ọjọ 31, Ọdun 2020: Ti gba wọle si ile-iwosan wa.

- Ilana ẹjẹ: WBC 3.99 x 10^9/L, HGB 66 g/L, PLT 57 x 10^9/L

- Iwọn fifun ẹjẹ agbeegbe: 69%

- Mofoloji ọra inu egungun: 90% awọn lymphoblasts ti ko dagba (awọn aruwo)

- Immunophenotyping: 84.07% ti awọn sẹẹli ti n ṣalaye CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, CD24 ti o han ni apakan, CD15dim, ti o nfihan igbẹkẹgbẹ B lymphoblasts.

- Jiini Fusion: MLL-ENL fusion gene rere, PCR pipo 44.419%

- Iyipada jiini: rere iyipada KMT2D (orisun germline)

- Chromosome karyotype: 46, XX, del (1) (p36.1), del (1) (q31q42), del (11) (q13), t (11;19) (q23; p13.3), fikun ( 14) (q34), -17, +mar [7]/46, idem, t (3;16) (p21; p13.3) [1]/46, XX [13]

- PET-CT: Tan kaakiri iṣelọpọ ti iṣelọpọ ni gbogbo iho-ara ati ọra inu eegun, ifura giga ti ikọlu aisan lukimia; splenomegaly pẹlu iṣelọpọ agbara ti o pọ si, o ṣee ṣe pẹlu aisan lukimia.

- Ti ṣe puncture lumbar ati intrathecal chemotherapy lẹẹkan, ko si awọn ohun ajeji ti a rii ni awọn idanwo ti o jọmọ CSF.

Itọju:

- Ọsẹ meji ti kimoterapi VLP, agbeegbe ẹjẹ nfa 5% ni Oṣu Kini Ọjọ 18.

- January 25: Agbeegbe ẹjẹ blasts 91%, mu pẹlu CTX, Ara-C, 6-MP kimoterapi.

- Kínní 3: Agbeegbe ẹjẹ awọn bugbamu 22%.

- Kínní 4: Gbigba ti 50ml autologous agbeegbe ẹjẹ fun CD19-CART cell asa.

- MTX 1g, FC chemotherapy (aisan 15mg lojoojumọ x 3 ọjọ, CTX 0.12g ojoojumo x 3 ọjọ).

- Kínní 13 (iṣaaju-idapo): Mofoloji ọra inu egungun fihan 87.5% blasts, ṣiṣan cytometry fihan 79.4% awọn bugbamu buburu.

- MLL-ENL idapọ pipo pupọ: 42.639%.

- Kínní 14: Idapo awọn sẹẹli CART ni iwọn 5 x 10 ^ 5 / kg.

- Awọn ipa ikolu ti o ni ibatan CAR-T: Ite 1 CRS (iba), ko si neurotoxicity.

- Ọjọ 20 lẹhin idapo: smear ẹjẹ fihan itọsi tumo, ipin sẹẹli CART 0.07%.

- Ailokun CART cell ailera.

- Oṣu Kẹta Ọjọ 8, Ọdun 2021: Ilana Ẹjẹ: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.

- Awọn bugbamu ẹjẹ agbeegbe: 83%. Ẹjẹ agbeegbe aifọwọyi ti a gba 60ml fun aṣa sẹẹli meji CART CD19/CD22.

- Ti ṣe itọju pẹlu cytarabine ati dexamethasone lati ṣakoso ẹru tumo.

- Oṣu Kẹta 18: FC chemotherapy (aisan 15mg lojoojumọ x 3 ọjọ, CTX 0.12g lojoojumọ x 3 ọjọ).

- March 22 (ṣaaju-idapo): Ẹjẹ ilana: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. Agbeegbe ẹjẹ mofoloji: 6% blasts.

- Egungun morphology: 91% blasts. Ti o ku ninu ọra inu egungun: 88.61% awọn sẹẹli ti n ṣalaye CD38, CD19, cCD79a, CD81, CD22, ti o nfihan igbẹkẹgbẹ B lymphoblasts.

- MLL-ENL idapọ pipo pupọ: 62.894%.

- Chromosome karyotype onínọmbà: 46, XX, del (1) (p36.1), del (11) (q13), t (11;19) (q23; p13.3), afikun (14) (q34), - 17, + Oṣù [2]/46, XX, del (1) (p36.1), del (1) (q31q42), del (11) (q13), t (11;19) (q23; p13.3). ), afikun (14) (q34).

- Oṣu Kẹta Ọjọ 23: Idapo awọn sẹẹli CART ni iwọn lilo 3 x 10 ^ 5 / kg.

- Oṣu Kẹta Ọjọ 26 siwaju: Iba giga ti o tẹsiwaju ati idagbasoke edema ti eto.

- Oṣu Kẹta Ọjọ 29: Mofoloji ẹjẹ agbeegbe: 92% awọn bugbamu; awọn transaminases ti o ga ati bilirubin.

- Oṣu Kẹrin Ọjọ 2: Ibẹrẹ ti ikọlu, ti a tọju pẹlu diazepam.

- Oṣu Kẹrin Ọjọ 2 (Ọjọ 10): Bibẹrẹ itọju methylprednisolone fun awọn ọjọ 3.

- Idahun CRS: Ipele 3, CRES: Ite 3.

- Oṣu Kẹrin Ọjọ 8 (Ọjọ 16): Ayẹwo ọra inu egungun fihan idariji morphological pipe, ṣiṣan cytometry odi fun awọn bugbamu buburu; Ìtúpalẹ̀ ìpilẹ̀ àbùdá ìdapọ̀ MLL-ENL: 0.