Clinical laboratory · 2025
Clinical Features and Prognosis Analysis of Acute Myeloid Leukemia in Children with DEK-CAN-Positive.
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Li Jiajia, Yuan Xi, Liang Chun, Zhang Lei, Li Zhen
Аннотация
BACKGROUND: The goal of the study is to investigate the clinical characteristics and prognostic analysis of acute myeloid leukemia in children with positive DEK-CAN fusion gene.
METHODS: The clinical characteristics and prognostic analysis methods of a case of acute myeloid leukemia in children with positive DEK-CAN fusion gene were retrospectively analyzed, and the domestic and international literature was reviewed.
RESULTS: The patient is a girl, 11 years old. The clinical diagnosis was acute myeloid leukemia M2a. Liver full, spleen large, a few small lymph nodes in bilateral axilla and retroperitoneum. Blood routine: WBC: 34.49 x 10⁹/L, RBC: 0.91 x 10¹²/L, Hb: 30g/L, Plt: 26 x 10⁹/L, the proportion of leukocyte classification granulocyte was significantly increased, and the primitive naive granulocyte accounted for 56%. Bone marrow smear: primitive naive myelodysplasia, 74% of myeloid original cells, large cell body, moderate plasma volume, round or irregular nuclei, fine nuclear chromatin, visible nucleoli. Peroxidase (MPO) staining: positive. Immunophenotypic expression of antigens CD117, HLA-DR, CD13, CD33, CD123, partial expression of antigens CD34, CD38, abnormal myeloid original cells. Patients tested by fusion gene were positive for DEK-CAN with FLT3-ITD mutation. The clinical di-agnosis was acute myeloid leukemia M2a. Chromosome karyotype analysis showed no split phase. The IA regimen, FLAG regimen, and HIA regimen were given successively, and no remission was achieved.
CONCLUSIONS: Patients with DEK-CAN fusion gene positive AML have a very poor prognosis, low primary induced remission rate, and high mortality. For confirmed cases, patients in remission with chemotherapy should undergo allogeneic hematopoietic stem cell transplantation as soon as possible to have a chance of long-term survival.
METHODS: The clinical characteristics and prognostic analysis methods of a case of acute myeloid leukemia in children with positive DEK-CAN fusion gene were retrospectively analyzed, and the domestic and international literature was reviewed.
RESULTS: The patient is a girl, 11 years old. The clinical diagnosis was acute myeloid leukemia M2a. Liver full, spleen large, a few small lymph nodes in bilateral axilla and retroperitoneum. Blood routine: WBC: 34.49 x 10⁹/L, RBC: 0.91 x 10¹²/L, Hb: 30g/L, Plt: 26 x 10⁹/L, the proportion of leukocyte classification granulocyte was significantly increased, and the primitive naive granulocyte accounted for 56%. Bone marrow smear: primitive naive myelodysplasia, 74% of myeloid original cells, large cell body, moderate plasma volume, round or irregular nuclei, fine nuclear chromatin, visible nucleoli. Peroxidase (MPO) staining: positive. Immunophenotypic expression of antigens CD117, HLA-DR, CD13, CD33, CD123, partial expression of antigens CD34, CD38, abnormal myeloid original cells. Patients tested by fusion gene were positive for DEK-CAN with FLT3-ITD mutation. The clinical di-agnosis was acute myeloid leukemia M2a. Chromosome karyotype analysis showed no split phase. The IA regimen, FLAG regimen, and HIA regimen were given successively, and no remission was achieved.
CONCLUSIONS: Patients with DEK-CAN fusion gene positive AML have a very poor prognosis, low primary induced remission rate, and high mortality. For confirmed cases, patients in remission with chemotherapy should undergo allogeneic hematopoietic stem cell transplantation as soon as possible to have a chance of long-term survival.