Disseminated intravascular coagulation score evolution in 48 h predicts early death in acute promyelocytic leukemia patients.

INTRODUCTION: Early death (ED) is the unsolved issue of acute promyelocytic leukemia (APL). The disseminated intravascular coagulation (DIC) score has been proposed as a marker of bleeding and death in APL; whether its temporal evolution predicts outcomes in APL is unknown. We evaluated whether an increasing score 48 h after diagnosis associates with ED. METHODS: Retrospective, single-center study, including patients with newly diagnosed APL between 2000 and 2023, treated with all-transretinoic acid (ATRA) plus anthracycline or arsenic trioxide (ATO). "DIC score worsening" was defined as ≥1 point increase in the score after 48 h, and ED as death within 30 days of diagnosis. RESULTS: Eighty-six patients were included, with median age of 46 years (17-82). ED patients (26.7%) more frequently had age >60 years and worsening DIC score after 48 h. These were also the only predictors of ED identified in both univariate and multivariate (OR 4.18, p = .011; OR 7.8, p = .005, respectively) logistic regression analysis. CONCLUSION: This is the first study on DIC score evolution in APL-a worsening DIC score 48 h after diagnosis is a strong independent predictive factor of ED. We propose a reduction of the DIC score from diagnosis as a new treatment goal in APL care.

Predictors of very early death in acute promyelocytic leukemia: a retrospective real-world cohort study.

Early death (ED) is still the major obstacle to cure in acute promyelocytic leukemia (APL). Most studies focus on 30-day ED; however, little is known on predictors of death before starting APL treatment (very early death - VED) and on predictors of 7-day ED, the period with most deaths due to thrombohemorrhagic diathesis. We hypothesized whether the severity of the coagulopathy of APL could predict VED and 7-day ED. We also aimed to evaluate other characteristics associated with these outcomes. We undertook a retrospective, single-center observational study including newly diagnosed APL patients admitted to our institution between January 2000 and November 2022. Baseline demographical, clinical, and laboratorial data were collected. Statistical analysis was performed using Stata. One hundred four patients were included. The VED rate was 4.8%. A DIC Score ≥ 7 (p = 0.045), serum creatinine > 1.5 mg/dL (p < 0.001%), a DIC Score ≥ 6 within 24 h (p = 0.009), and mechanical ventilation (p < 0.001) were associated with VED. The 7-day ED rate was 12.5%. High-risk (p = 0.007) and hypogranular APL (p = 0.029), DIC Score at diagnosis (p = 0.047), DIC Score ≥ 7 (p = 0.043), DIC Score ≥ 6 within 24 h (p = 0.025), PT prolongation > 6 s (p = 0.002), and creatinine > 1.5 mg/dL (p = 0.004) were associated with 7-day ED. However, only elevated creatinine emerged as an independent predictor of 7-day ED (OR 21.4; p = 0.008). Our study shows that in patients with APL, an elevated creatinine at diagnosis strongly predicts for 7-day ED. A DIC Score ≥ 7 and a Score that remains ≥ 6 within 24 h and a serum creatinine > 1.5 mg/dL significantly associated with VED.

Chronic myeloproliferative diseases.

The chronic myeloproliferative disorders are a group of diseases in which there is an increased proliferation of one or more subtypes of myeloid cells; they include essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF). In ET and PV the main neurologic manifestations are headaches, dizziness and macro- and microvascular, both venous and arterial, thrombosis and intracranial hemorrhages. Paresthesias and chorea also occur in PV. In PMF neurologic complications are very rare and consist predominantly of spinal cord compression by extramedullary hematopoiesis tissue.