Aisan lukimia Lymphoblastic nla (T-ALL) -01

Awọn ẹya ile-iwosan:

- Ayẹwo: T-cell lymphoblastic lymphoma/leukemia

- Oṣu Kẹta 2020: Ti gbekalẹ pẹlu Ikọaláìdúró paroxysmal ati ọpọ mediastinal, timo T-cell lymphoblastic lymphoma nipasẹ mediastinal mass puncture biopsy.

- Ti gba awọn akoko 8 ti kimoterapi ati ju awọn akoko 20 ti itọju redio, ti o yọrisi idinku nla ti ibi-aarin aarin.

- Oṣu Kini Ọjọ 16, Ọdun 2021: Idagbasoke irora ni apa ọtun isalẹ.

- Ilana ẹjẹ: WBC 122.29 x 10^9/L, HGB 91 g/L, PLT 51 x 10^9/L

- Ẹjẹ-ara-ara-ara-ara: 95.5% awọn lymphoblasts akọkọ.

- Sitometry sisan ọra inu egungun: 91.77% awọn sẹẹli jẹ lymphoblasts T-cell ti ko dagba.

- Jiini lesese: Awọn iyipada ni NOTCH1, IL7R, ASXL2 Jiini ri.

- Gbigba Hyper-CVAD/B ilana, ilana ESHAP ni atẹle, mejeeji ko ni doko pẹlu iba ti o tẹsiwaju.

- Kínní 18, 2021: Ti gba wọle si ile-iwosan wa.

- Ti gbekalẹ pẹlu iba, àyà CT fihan pneumonia.

- Ilana ẹjẹ: WBC 2.89 x 10^9/L, HGB 57.7 g/L, PLT 14.9 x 10^9/L

- Agbeegbe ẹjẹ awọn sẹẹli ti ko dagba: 90%

- Mofoloji ọra inu egungun: Hypercellular (Iwe IV), 85% awọn lymphoblasts akọkọ.

- Immunophenotyping: 87.27% ti awọn sẹẹli jẹ awọn lymphoblasts T-cell alakoko buburu.

- Ayẹwo Chromosomal: 46,XX [24]; meta afikun ajeji karyotypes woye.

- Awọn Jiini ti o yipada:

1. IL7R T244_I245insARCPL iyipada rere

2. NOTCH1 E1583_Q1584dup iyipada rere

3. ASXL2 Q602R iyipada rere

- Aworan jiini idapọmọra Lukimia: Odi

- Awọn abajade PET/CT: Ko si foci tumo hypermetabolic pataki ninu gbogbo egungun ati ọra inu eegun.

Itọju:

- Bibẹrẹ kimoterapi ijọba VP, alaye bi atẹle: Vincristine (VDS) 3mg lẹẹkan, Dexamethasone (Dex) 7mg ni gbogbo wakati 12 fun awọn ọjọ 9, pẹlu itọju egboogi-aisan.

- Oṣu Kẹta Ọjọ 1: Awọn sẹẹli ti ko dagba ni agbeegbe ti dinku si 7%.

- Oṣu Kẹta Ọjọ 4: Awọn lymphocytes autologous ti a kojọpọ fun aṣa sẹẹli CD7-CAR T.

- Oṣu Kẹta Ọjọ 8: Ilana VLP ti bẹrẹ ni idapo pẹlu itọju Sida benzamine.

- Oṣu Kẹta 14: Ti gba chemotherapy ijọba FC (Fludarabine 0.35g fun awọn ọjọ 3, Cyclophosphamide 45mg fun awọn ọjọ 3).

- Oṣu Kẹta Ọjọ 17 (idapo sẹẹli ṣaaju):

- Ajẹsara ajẹsara ti o ku: 15.14% awọn sẹẹli ṣe afihan imọlẹ CD7, CD3 dim, CD3 cytoplasmic, T cell receptor restricted delta (TCRrd), ikosile apa kan ti CD99, ti o nfihan awọn sẹẹli T ti o buruju.

- Oṣu Kẹta Ọjọ 19: Awọn sẹẹli CD7-CAR T autologous ti a fi sii (1 x 10 ^ 6 / kg).

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

- Oṣu Kẹrin Ọjọ 6 (Ọjọ 17): Mofoloji ọra inu egungun fihan idariji, cytometry ṣiṣan ko ṣe awari awọn sẹẹli alakoko buburu.