High bleeding rates in δ-storage pool disease during surgeries and deliveries.

BACKGROUND

δ-storage pool disease (δ-SPD) involves various platelet disorders due to deficient or reduced secretion of dense granules, causing increased bleeding tendency, particularly during surgery and delivery. The optimal treatment to prevent bleeding in δ-SPD is unknown.

OBJECTIVES

To evaluate the efficacy of preventive treatment in a cohort of patients with δ-SPD.

METHODS

In a single-center study, we retrospectively reviewed the medical files of δ-SPD patients diagnosed between 2016 and 2024. Data included invasive diagnostic procedures, surgeries (minor/major), and deliveries (vaginal/cesarean section) before and after diagnosis. Diagnosis was confirmed by platelet adenosine diphosphate levels (<1.4 μmol/1011 platelets). Bleeding complications were defined according to the International Society on Thrombosis and Haemostasis bleeding scale. Data on treatment and platelet transfusions were documented.

RESULTS

Thirty-eight patients (mean age, 35.6 years; range, 0-67; 63.2% female) were included. The average adenosine diphosphate level was 0.82 μmol/1011 platelets (range, 0.2-1.4). A total of 161 interventions and deliveries were analyzed. The bleeding rate was dependent on the timing of diagnosis (51.5% before δ-SPD diagnosis and 16.8% after; P < .001) and on the use of preventive treatment (45.8% without and 17.9% with; P < .001). A total of 145 interventions were analyzed, including 63 minor and 82 major. Of these, 43/145 (29.7%) were complicated by bleeding (39 minor bleeds and 4 major bleeds). Sixteen deliveries were analyzed. Among 10 vaginal deliveries, 9 had postpartum hemorrhage, of whom 6 had no prophylaxis. All 6 cesarean sections received prophylactic platelet transfusion; no bleeding occurred.

CONCLUSION

We observed a high perioperative/peripartum bleeding rate in patients with δ-SPD. This decreased significantly after a correct diagnosis. In addition, bleeding rates were significantly lower among patients who received preventive treatment before the procedure.