Aisan lukimia Lymphoblastic nla (T-ALL) -02

Awọn ẹya ara ẹrọ iwosan:

- Ayẹwo: Ẹjẹ-ẹjẹ-ẹjẹ-ẹjẹ-ẹjẹ T-cell

- Bibẹrẹ: Oṣu Kẹta Oṣu Kẹta ọdun 2018

- Awọn aami aiṣan akọkọ: Ọpọ ọpọ ọra-ọpa-ara ti o gbooro jakejado ara

- Iṣe deede ẹjẹ akọkọ: WBC: 39.46*10^9/L, Hb: 129g/L, PLT: 77*10^9/L

- Egungun morphology: 92% blasts

-Sitometry sisan: 95.3% awọn sẹẹli ajeji ti n ṣalaye

TdT+CD99+CyCD3+CD7stCd5DdimCD4-CD8-mCD3-CD45dim

- Fusion Jiini: odi

- Jiini iyipada: NOTCH1 jiini iyipada ri

- Itupalẹ Chromosome: Karyotype deede

Itan itọju:

- Oṣu Kẹrin Ọjọ 3, Ọdun 2018: Itọju ifakalẹ pẹlu ilana VDCP

- Oṣu Kẹrin Ọjọ 18, Ọdun 2018: Awọn ikọlu ọra inu egungun ni 96%

- Oṣu Kẹrin Ọjọ 20, Ọdun 2018: Aṣeyọri idariji lẹhin ilana ilana CAG

- Oṣu Karun ọjọ 18, Ọdun 2018: Itọju imudarapọ pẹlu ilana ilana CMG+VP

- Oṣu Kẹfa Ọjọ 22, Ọdun 2018: Awọn fifun ọra inu egungun pọ si 40%, ifasẹyin ti aisan lukimia

- Oṣu Keje Ọjọ 25, Ọdun 2018: Ilana CLAM (clarithromycin+cyclophosphamide+amikacin)

- Iṣipopada sẹẹli hematopoietic lati ọdọ arakunrin ti o baamu HLA ni lilo imudara FLU + BU ni Oṣu Kẹjọ Ọjọ 14

- Abojuto lẹhin asopo-pada: idariji ti iṣan ara eegun ni oṣu 1, oṣu 3, oṣu 6, oṣu 9, ati oṣu 11

- Mofoloji ọra inu egungun fihan idariji ni awọn oṣu 16 lẹhin isọdọmọ, pẹlu cytometry ṣiṣan ti n ṣafihan 0.02% awọn lymphocytes ti ko dagba.

- Oṣu kọkanla ọjọ 13, Ọdun 2020: Chimerism ẹjẹ agbeegbe lati orisun oluranlọwọ jẹ 97.9%

Awọn sẹẹli akọkọ ti ẹjẹ agbeegbe: 20%

- Oṣu kejila ọjọ 18, Ọdun 2020: Mofoloji ọra inu egungun: 60.6% awọn bugbamu

- Sitometry sisan: 30.85% awọn lymphocytes T ti ko dagba buburu

- Itupalẹ chromosome: 46, XY (20)

- Ti gba DA kimoterapi ilana ni Oṣu Kini Ọjọ 19, Ọdun 2021

- Mofoloji ọra inu egungun ni Oṣu Kini Ọjọ 19, Ọdun 2021: hyperplasia Ite III, 16% awọn bugbamu

- Ayẹwo karyotype chromosome: 46, XY (20)

Sitometry ṣiṣan: 7.27% ti awọn sẹẹli (laarin awọn sẹẹli iparun) ti ṣafihan CD99bri, CD13, CD38, cbcl-2, cCD3, HLA-ABC bri, CD7bri, ati CD5dim ni apakan ti a fihan, ti n tọka si awọn lymphocytes T ti ko dagba.

- Aworan jiini idapọmọra Lukimia: Odi

- Iṣiro iyipada tumọ ẹjẹ (awọn oriṣi 86):

1. PHF6 K299Efs * 13 iyipada rere

2. RUNX1 S322 * iyipada rere

3. FBXW7 E471G iyipada rere

4. JAK3 M511I iyipada rere

5. NOTCH1 Q2393 * iyipada rere

Itọju:

- January 22: Gbigba ati asa ti autologous agbeegbe ẹjẹ lymphocytes fun CD7-CART

- Ṣaaju si idapo CD7-CART, alaisan gba VLP (vincristine, l-asparaginase, prednisone) pẹlu bortezomib chemotherapy.

- Kínní 3: kimoterapi ijọba FC (aisan 50mg fun awọn ọjọ 3 + CTX 0.45g fun awọn ọjọ 3)

- Kínní 5 (iṣaaju-idapo): Ẹkọ-ara-ara ti egungun fihan 23% blasts.

Sitometry ṣiṣan ti ṣafihan awọn sẹẹli 4.05% ti n ṣalaye CD99bri, CD5dim, CD7bri, TDT, cCD3, ti n tọka si awọn lymphocytes T ti ko dagba.

- Itupalẹ Chromosome: 46, XY (20)

- Itupalẹ Chimerism (post-HSCT): Awọn sẹẹli ti o jẹ oluranlọwọ ṣe iṣiro fun 52.19%.

- Kínní 7: Idapo awọn sẹẹli CD7-CART autologous ni iwọn lilo 5 * 10 ^ 5 / kg.

- Kínní 15: Agbeegbe ẹjẹ awọn sẹẹli ti ko dagba dinku si 2%.

- Kínní 19 (Ọjọ 12 lẹhin idapo): Alaisan ni iba, eyiti o duro fun awọn ọjọ 5 ṣaaju iṣakoso iwọn otutu ti waye.

- Oṣu Kẹta Ọjọ 2: Ayẹwo ọra inu egungun fihan idariji ti ara pipe, pẹlu cytometry ṣiṣan ti kii ṣe iwari awọn sẹẹli ti ko dagba.