Case 2
A female pediatric patient aged 3 years and 7 months was hospitalized after intermittent fever for 10 days in May 2020. TDT, CD10, CD19, CD22, CD34, HLA-DR, and Ccd79a expressions were identified in bone marrow aspirate by flow cytometry. The BCR-ABL fusion gene was shown to be positive by FISH and reverse transcription polymerase chain reaction (RT-PCR). Karyotype analysis of the pediatric patient revealed chromosomal translocation with t(9;22) (q34; q11), and the diagnosis was Ph+ B-ALL. A chemotherapy regimen combined with oral imatinib was administered according to the CCLG-ALL-2018 protocol[6]. After 1 month of treatment,BCR-ABL was quantified as 2.9%, and the results for minimal residual disease in the bone marrow were negative, so the TKI regimen was changed to oral dasatinib. The BCR-ABL fusion gene in the patient’s bone marrow did not show complete remission after 5 months of sequential chemotherapy. The patient underwent related-donor haploid allogeneic hematopoietic stem cell transplantation, with the donor being the patient’s biological father. The bone marrow reconstitution was successful after the allo-HSCT, and the patient continued to take dasatinib orally for maintenance therapy. Periodic routine examination indicated negative results for the BCR-ABL fusion gene and minimal residual disease in the bone marrow, and the results of cardiac ultrasound during the therapy period were normal.
Nevertheless, the patient’s echocardiography showed enlargement of the right cardiac cavities, widening of the pulmonary artery, moderate to severe tricuspid regurgitation, and pulmonary hypertension in January 2022 (after treatment with dasatinib for 18 months). Dasatinib was promptly discontinued, and oral captopril was simultaneously started for treating the PAH. Echocardiography reexaminations demonstrated slightly expanded right cardiac cavities, a slightly widened pulmonary artery, moderate tricuspid regurgitation, and a PASP of 28 mmHg (Table 2 and Figure 1). The patient is currently receiving oral consolidation therapy with captopril, and echocardiography showed that the PASP was not elevated.