Khansa ya m'magazi ya Lymphoblastic (B-ALL)-02

Makhalidwe a Mlandu:

- Meyi 19, 2019: Anapezeka ndi khansa ya m'magazi ya B-cell lymphoblastic (B-ALL)

- Amawonetsedwa ndi ma multiple scalp masses ndi lymphadenopathy

- Kuchuluka kwa magazi m'magazi: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, ma lymphocyte osazolowereka 4%

- Kapangidwe ka mafupa: 80.2% ma lymphoblast osakhwima (kuphulika)

- Kuchiza matenda a chitetezo chamthupi: 74.19% ya maselo ndi maselo oyambitsa matenda a B-lineage omwe amaonetsa CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, omwe amaonetsa pang'ono cIgM. Kuzindikira: B-ALL (Gawo la Pre-B)

- Jini yosakanikirana: MLL-ENL positive, Philadelphia chromosome-like (Ph-like) screen negative

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

- Chithandizo cha VDLD regimen chemotherapy poyamba chinapeza chikhululukiro cha chitetezo chamthupi patatha mwezi umodzi, MLL-ENL quantitative PCR 0.026%

- Kupitiliza kulandira mankhwala a chemotherapy motsatira ndondomeko ya ana, MLL-ENL quantitative PCR 0 pambuyo pa nthawi yachinayi. Mankhwala ena a chemotherapy adapitilira.

- Marichi 2020: Matenda otsalira a immunological immunological 0.35%, MLL-ENL quantitative PCR 0.53%, zomwe zikusonyeza kuti matendawa akubwereranso. Banja linakana kuikidwanso. Anapitiriza kulandira mankhwala a chemotherapy kwa maulendo atatu.

- Julayi 2020: Mafupa anabwereranso mwakale.

- Novembala 11, 2020: Mankhwala a intrathecal chemotherapy, matenda otsalira a immunological a CSF 66%, adapezeka ndi khansa ya m'mitsempha yapakati. Mankhwala a intrathecal chemotherapy adabwerezedwa kawiri, CSF idasanduka negative.

- Disembala 31, 2020: Analowa m'chipatala chathu.

- Kuchuluka kwa magazi: WBC 3.99 x 10^9/L, HGB 66 g/L, PLT 57 x 10^9/L

- Chiwerengero cha magazi ophulika m'mbali mwa thupi: 69%

- Kapangidwe ka mafupa: 90% ya ma lymphoblast osakhwima (kuphulika)

- Kujambula kwa chitetezo chamthupi: 84.07% ya maselo amaonetsa CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, amaonetsa pang'ono CD24, CD15dim, zomwe zikusonyeza kuti ma lymphoblast a B osakhwima ndi owopsa.

- Jini yosakanikirana: MLL-ENL jini yosakanikirana ndi yabwino, kuchuluka kwa PCR 44.419%

- Kusintha kwa majini: Kusintha kwa KMT2D komwe kwapezeka (komwe kunachokera)

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

- PET-CT: Kuchuluka kwa kagayidwe kachakudya m'mafupa onse ndi m'mafupa, kukayikira kwakukulu kuti khansa ya m'magazi idzabwereranso; splenomegaly yokhala ndi kagayidwe kachakudya kowonjezereka, komwe mwina kumakhudza khansa ya m'magazi.

- Ndinachita kuboola lumbar ndi intrathecal chemotherapy kamodzi, palibe zolakwika zomwe zinapezeka mu mayeso okhudzana ndi CSF.

Chithandizo:

- Masabata awiri a VLP chemotherapy, magazi ozungulira amawonjezeka ndi 5% pa Januware 18.

- Januwale 25: Magazi otuluka m'magazi a m'mbali mwa thupi adaphulika ndi 91%, ndipo adalandira chithandizo cha CTX, Ara-C, 6-MP chemotherapy.

- February 3: Kuphulika kwa magazi m'mbali mwa thupi ndi 22%.

- February 4: Kusonkhanitsa magazi a 50ml a autologous peripheral kuti apeze maselo a CD19-CART.

- MTX 1g, FC chemotherapy (Flu 15mg tsiku lililonse x masiku atatu, CTX 0.12g tsiku lililonse x masiku atatu).

- February 13 (asanalowe m'thupi): Kapangidwe ka mafupa a m'mafupa kamasonyeza 87.5% ya kuphulika, ndipo kayendedwe ka madzi kamasonyeza 79.4% ya kuphulika kwa malignant.

- Kusanthula kwa kuchuluka kwa majini a MLL-ENL: 42.639%.

- February 14: Kulowetsedwa kwa maselo a CART pa mlingo wa 5 x 10^5/kg.

- Zotsatira zoyipa zokhudzana ndi CAR-T: CRS ya Giredi 1 (malungo), palibe poizoni muubongo.

- Tsiku la 20 pambuyo pa kulowetsedwa: Kuyeza magazi kukuwonetsa kuchuluka kwa chotupa, kuchuluka kwa maselo a CART ndi 0.07%.

- Chithandizo cha maselo a CART chosagwira ntchito bwino.

- Marichi 8, 2021: Kuyeza magazi: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.

- Kuphulika kwa magazi m'mbali: 83%. Magazi ozungulira okha anasonkhanitsidwa 60ml kuti apeze maselo awiri a CD19/CD22 a CART.

- Amalandira mankhwala a cytarabine ndi dexamethasone kuti achepetse kuchuluka kwa chotupa.

- Marichi 18: FC chemotherapy (Chimfine 15mg tsiku lililonse x masiku atatu, CTX 0.12g tsiku lililonse x masiku atatu).

- Marichi 22 (musanayambe kumwa): Kuyeza magazi: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. Kapangidwe ka magazi ozungulira: 6% kuphulika.

- Kapangidwe ka mafupa: 91% ya kuphulika. Zotsalira mu mafupa: 88.61% ya maselo omwe ali ndi CD38, CD19, cCD79a, CD81, CD22, zomwe zikusonyeza ma lymphoblasts a B osakhwima.

- Kusanthula kwa kuchuluka kwa majini a MLL-ENL: 62.894%.

- Kusanthula kwa karyotype ya chromosome: 46, XX, del(1)(p36.1), del(11)(q13), t(11;19)(q23;p13.3), onjezerani(14)(q34), -17, +mar [2]/46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3), onjezerani(14)(q34).

- Marichi 23: Kulowetsedwa kwa maselo a CART pa mlingo wa 3 x 10^5/kg.

- 26 Marichi kupita mtsogolo: Malungo amphamvu osalekeza ndipo kenako anayamba kutupa m'thupi.

- Marichi 29: Kapangidwe ka magazi m'thupi: 92% ya kuphulika; kuchuluka kwa ma transaminases ndi bilirubin.

- Epulo 2: Kuyamba kwa khunyu, kuchiritsidwa ndi diazepam.

- Epulo 2 (Tsiku 10): Anayamba kulandira mankhwala a methylprednisolone kwa masiku atatu.

- CRS reaction: Giredi 3, CRES: Giredi 3.

- Epulo 8 (Tsiku 16): Kuwunika kwa mafupa kukuwonetsa kuchotsedwa kwathunthu kwa mawonekedwe, flow cytometry negative kwa malignant blasts; MLL-ENL fusion gene quantitative analysis: 0.