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.