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Abstract Background Early death remains a major factor in survival in APL. We aimed to analyze the risk factors for differentiation syndrome and early death in acute promyelocytic leukemia (APL). Methods The clinical data of APL patients who were newly diagnosed at Mianyang Central Hospital from January 2013 to January 2022 were retrospectively analyzed. Results Eighty-six newly diagnosed APL patients (37 males and 49 females) were included in this study. The median age was 46 (17–75) years. Sixty-one patients (70.9%) had low/intermediate-risk APL, and 25 patients (29.1%) had high-risk APL. The incidence of differentiation syndrome (DS) was 62.4%. The multivariate analysis showed that a peak white blood cell (WBC) count ≥16 × 10^9/L was an independent risk factor (OR = 11.000, 95% CI: 2.830–42.756, P = 0.001) for DS in all APL patients, while a WBC count ≥10 × 10^9/L on Day 5 was an independent risk factor for DS in low-intermediate risk APL patients (OR = 9.114, 95% CI: 2.384–34.849, P = 0.001). There were 31 patients (36.5%) with mild DS and 22 patients (25.9%) with severe DS. The multivariate analysis showed that WBC count ≥23 × 10^9/L at chemotherapy was an independent risk factor for severe DS (OR = 10.500, 95% CI: 2.344–47.034, P = 0.002). The rate of early death (ED) was 24.4% (21/86). The multivariate analysis showed that male gender (OR = 7.578,95% CI:1.136–50.551, P = 0.036), HGB 7 × 10^9/L on Day 3 have a higher risk of ED. Leukocyte proliferation is associated with TNF-α rather than IL-6, and TNF-α may be a potential biomarker for predicting ED.

Objective To investigate the molecular biological changes of relapsed acute promyelocytic leukemia (APL), and to provide guidance for the clinical diagnosis and treatment of relapsed APL. Methods A retrospective analysis was performed on the clinical data of a pediatric patient with relapsed APL who was molecular relapse 8 months after drug withdrawal, and related literature was reviewed. Results The patient was a 1-year-old boy who initially presented with fever and hemorrhagic spots on the skin, and physical examination showed the appearance of anemia. The skin and mucosa of his whole body were covered with hemorrhagic spots and ecchymosis. Several enlarged lymph nodes that had fused together could be palpated on the left side of the neck, and the liver was palpable at 2 cm below the costal margin. The routine blood test showed low hemoglobin and platelet counts. The bone marrow smear showed that the proportion of promyelocytes was 75%, and POX was positive. The polymerase chain reaction and fluorescence in situ hybridization assay showed positive results for the PML/RARα fusion gene in the bone marrow smear. NRAS gene mutation was detected, and the initial diagnosis was APL. Following standard therapy, the patient’s bone marrow smear and minimal residual disease showed complete remission, and the PML/RARα fusion gene was no longer detectable. However, 8 months after drug withdrawal, the reexamination revealed that the L-type PML/RARα in the bone marrow of the child accounted for 4.51%. Therefore, the child was diagnosed with molecular relapse of APL and was given a chemotherapy regimen against relapsed APL. As a result, the PML/RARα of the child turned negative. Afterwards, the regular chemotherapy was continued. Meanwhile, autologous stem cell collection and regular reexamination were performed. Conclusion For relapsed APL, once molecular relapse is confirmed, treatment should be started as soon as possible, and regular detection of the PML/RARα fusion gene should be performed. Moreover, hematopoietic stem cell transplantation should be considered if second molecular remission cannot be achieved.

Background: Early death remains a major factor in survival in APL. We aimed to analyze the risk factors for differentiation syndrome and early death in acute promyelocytic leukemia (APL). Methods: The clinical data of APL patients who were newly diagnosed at Mianyang Central Hospital from January 2013 to January 2022 were retrospectively analyzed. Results: Eighty-six newly diagnosed APL patients (37 males and 49 females) were included in this study. The median age was 46 (17–75) years. Sixty-one patients (70.9%) had low/intermediate-risk APL, and 25 patients (29.1%) had high-risk APL. The incidence of differentiation syndrome (DS) was 62.4%. The multivariate analysis showed that a peak white blood cell (WBC) count ≥16 × 10^9/L was an independent risk factor (OR = 11.000, 95% CI: 2.830–42.756, P = 0.001) for DS in all APL patients, while a WBC count ≥10 × 10^9/L on Day 5 was an independent risk factor for DS in low-intermediate risk APL patients (OR = 9.114, 95% CI: 2.384–34.849, P = 0.001). There were 31 patients (36.5%) with mild DS and 22 patients (25.9%) with severe DS. The multivariate analysis showed that WBC count ≥23 × 10^9/L at chemotherapy was an independent risk factor for severe DS (OR = 10.500, 95% CI: 2.344–47.034, P = 0.002). The rate of early death (ED) was 24.4% (21/86). The multivariate analysis showed that male gender (OR = 7.578,95% CI:1.136–50.551, P = 0.036), HGB < 65 g/L (OR = 16.271,95% CI:2.012–131.594, P = 0.009) and WBC count ≥7 × 10^9/L on Day 3(OR = 23.359,95% CI:1.825–298.959, P = 0.015) were independent risk factors for ED. The WBC count at diagnosis, WBC count on Day 3 and WBC count on Day 5 had moderate positive correlations with tumor necrosis factor-α (TNF-α) at diagnosis, and the correlation coefficients were 0.648 (P = 0.012), 0.615 (P = 0.033), and 0.609 (P = 0.035), respectively. The WBC count had no correlation with IL-6. Conclusion: During induction treatment, cytotoxic chemotherapy may need to be initiated to reduce the risk of DS for APL patients with a low-intermediate risk WBC count ≥10 × 10^9/L on Day 5 or for all patients with a peak WBC count ≥16 × 10^9/L. Patients with WBC > 7 × 10^9/L on Day 3 have a higher risk of ED. Leukocyte proliferation is associated with TNF-α rather than IL-6, and TNF-α may be a potential biomarker for predicting ED. [ABSTRACT FROM AUTHOR]