Ọrịa kansa ọbara Lymphoblastic dị oke njọ (B-ALL)-02

Njirimara Ikpe:

- Mee 19, 2019: Achọpụtara ya na ọrịa leukemia lymphoblastic nke mkpụrụ ndụ B (B-ALL)

- Na-egosi ọtụtụ ọnya isi na lymphadenopathy

- Usoro ọbara: WBC 13.3 x 10^9/L, HGB 94 g/L, PLT 333 x 10^9/L, lymphocytes na-adịghị mma 4%

- Ọdịdị nke ụmị ọkpụkpụ: 80.2% lymphoblasts na-etobeghị eto (gbawara agbawa)

- Immunophenotyping: 74.19% nke mkpụrụ ndụ bụ mkpụrụ ndụ B-lineage dị njọ nke na-egosipụta CD45dim, CD19, CD9, CD22, CD81, CD58, cCD79a, CD38, HLA-DR, na-egosipụta cIgM n'akụkụ ụfọdụ. Nchọpụta: B-ALL (Ọkwa tupu B)

- Mkpụrụ ndụ ihe nketa njikọta: MLL-ENL dị mma, ihuenyo Philadelphia dị ka kromosome (dị ka Ph) adịghị mma

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

- Usoro ọgwụgwọ VDLD nwetara mbelata mgbochi ọrịa na mbụ mgbe otu ọnwa gasịrị, MLL-ENL quantitative PCR 0.026%

- Ọgwụ chemotherapy na-aga n'ihu dịka usoro ọgwụgwọ ụmụaka si dị, MLL-ENL quantitative PCR 0 mgbe okirikiri nke anọ gasịrị. Ọgwụ chemotherapy na-aga n'ihu.

- Machị 2020: Ọrịa fọdụrụ n'ime akwara ọkpụkpụ 0.35%, MLL-ENL quantitative PCR 0.53%, nke na-egosi etu esi agaghachi azụ. Ezinụlọ ekweghị ka e tinye ya n'ọrụ. A na-aga n'ihu na-agwọ ọrịa chemotherapy maka oge atọ.

- Julaị 2020: Ọkpụkpụ ahụ laghachiri azụ nke ọma.

- Nọvemba 11, 2020: Ọgwụ chemotherapy intrathecal, ọrịa residual immunological CSF 66%, achọpụtara na ọ nwere leukemia sistemu akwara etiti. Ọgwụ chemotherapy intrathecal ugboro abụọ, CSF gbanwere na-adịghị mma.

- Disemba 31, 2020: E tinyere ya n'ụlọ ọgwụ anyị.

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

- Ọnụọgụ ọbara gbara gburugburu: 69%

- Ọdịdị nke ụmị ọkpụkpụ: 90% lymphoblasts na-etobeghị eto (gbawara agbawa)

- Immunophenotyping: 84.07% nke mkpụrụ ndụ na-egosipụta CD38, CD19, CD81dim, cCD79a, HLA-DR, cIgM, CD22, CD123, na-egosipụta CD24, CD15dim n'akụkụ, na-egosi lymphoblast B na-etobeghị eto.

- Mkpụrụ ndụ ihe nketa njikọta: Mkpụrụ ndụ ihe nketa njikọta MLL-ENL dị mma, PCR ọnụọgụgụ 44.419%

- Mgbanwe mkpụrụ ndụ ihe nketa: Mmụpụta KMT2D dị mma (mmalite germline)

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

- PET-CT: Mmụba nke metabolism na-agbasa n'ime oghere ọkpụkpụ na ụmị ọkpụkpụ dum, nnukwu enyo na leukemia na-alọghachi azụ; splenomegaly na mmụba nke metabolism, o yikarịrị ka ọ gụnyere leukemia.

- Emere m ọkpọ akwara lumbar na chemotherapy n'ime ahụ otu ugboro, ọ dịghị ihe na-adịghị mma a hụrụ na ule ndị metụtara CSF.

Ọgwụgwọ:

- Izu abụọ nke ọgwụgwọ VLP, ọbara mpaghara na-agbawa pasent 5 na Jenụwarị 18.

- Jenụwarị 25: Ọbara gbara gburugburu 91%, ejiri ọgwụgwọ CTX, Ara-C, na 6-MP gwọọ ya.

- Febụwarị 3: Ọbara gbara gburugburu 22%.

- Febụwarị 4: Nchịkọta ọbara akụkụ 50ml maka mmepụta mkpụrụ ndụ CD19-CART.

- MTX 1g, FC chemotherapy (Flu 15mg kwa ụbọchị x ụbọchị 3, CTX 0.12g kwa ụbọchị x ụbọchị 3).

- Febụwarị 13 (tupu a gbanye): Ọdịdị ọkpụkpụ na-egosi mgbawa 87.5%, cytometry na-egosi mgbawa ọjọọ 79.4%.

- Nyocha ọnụọgụ mkpụrụ ndụ ihe nketa MLL-ENL: 42.639%.

- Febụwarị 14: Ntinye mkpụrụ ndụ CART na dose nke 5 x 10^5/kg.

- Mmetụta ọjọọ ndị metụtara CAR-T: Ọkwa 1 CRS (ahụ ọkụ), enweghị nsí na-adịghị mma.

- Ụbọchị nke iri abụọ mgbe a mịnyechara ya: Ọbara na-egosi mmụba nke etuto ahụ, oke mkpụrụ ndụ CART 0.07%.

- Ọgwụgwọ mkpụrụ ndụ CART na-adịghị arụ ọrụ nke ọma.

- Machị 8, 2021: Usoro ọbara: WBC 38.55 x 10^9/L, HGB 65g/L, PLT 71.60 x 10^9/L.

- Ọbara dị n'akụkụ: 83%. Ọbara dị n'akụkụ onwe ya anakọtara 60ml maka mkpụrụ ndụ CART abụọ nke CD19/CD22.

- A na-eji cytarabine na dexamethasone agwọ ọrịa ahụ iji chịkwaa ibu ọrịa kansa.

- Machị 18: Ọgwụ FC (Flu 15mg kwa ụbọchị x ụbọchị 3, CTX 0.12g kwa ụbọchị x ụbọchị 3).

- Machị 22 (tupu a gbanye ya): Usoro ọbara: WBC 0.42 x 10^9/L, HGB 93.70g/L, PLT 33.6 x 10^9/L. Ọdịdị ọbara dị n'akụkụ: 6% mgbawa.

- Ọdịdị nke ụmị ọkpụkpụ: 91% mgbawa. Ihe fọdụrụ n'ime ụmị ọkpụkpụ: 88.61% mkpụrụ ndụ na-egosipụta CD38, CD19, cCD79a, CD81, CD22, na-egosi lymphoblast B nke na-etobeghị eto.

- Nyocha ọnụọgụ mkpụrụ ndụ ihe nketa MLL-ENL: 62.894%.

- Nyocha karyotype kromosome: 46, XX, del(1)(p36.1), del(11)(q13), t(11;19)(q23;p13.3), tinye(14)(q34), -17, +mar [2]/46, XX, del(1)(p36.1), del(1)(q31q42), del(11)(q13), t(11;19)(q23;p13.3), tinye(14)(q34).

- Machị 23: Ntinye mkpụrụ ndụ CART na dose nke 3 x 10^5/kg.

- Machị 26 gaa n'ihu: Ahụ ọkụ na-adịgide adịgide ma mechaa nwee ọzịza sistemụ.

- Machị 29: Ọdịdị ọbara dị n'akụkụ: 92% mgbawa; mmụba nke transaminases na bilirubin.

- Eprel 2: Mmalite nke ọdịdọ, ejiri diazepam gwọọ ya.

- Eprel 2 (Ụbọchị nke 10): Amalitere ọgwụgwọ methylprednisolone ruo ụbọchị atọ.

- Mmeghachi omume CRS: Klas nke 3, CRES: Klas nke 3.

- Eprel 8 (Ụbọchị 16): Nyocha ọkpụkpụ na-egosi mwepụ zuru oke nke morphological, na cytometry na-adịghị mma maka mgbawa ọjọọ; MLL-ENL fusion gene quantitative analysis: 0.