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Variation in Platelet Delta Granules Over Time in Young Women Undergoing Evaluation for Heavy Menstrual Bleeding
Gary M Woods1, Natasha Pillay Smiley2, Joseph Stanek3
11 Division of Pediatric Hematology/Oncology, Children's Hospital of the King's Daughters, Norfolk, Virginia.
Insights
Platelet electron microscopy (EM) for delta-granule platelet storage pool deficiency (δ-PSPD) shows significant variability within patients. Caution is advised when using EM alone for δ-PSPD diagnosis due to inconsistent results.
Area of Science:
- Hematology
- Platelet Physiology
- Diagnostic Imaging
Background:
- Delta-granule platelet storage pool deficiency (δ-PSPD) is a platelet function defect causing bleeding issues.
- Platelet electron microscopy (EM) is a standard diagnostic tool for δ-PSPD.
- Intrapatient variability of platelet δ-granule counts by EM is not well understood.
Purpose of the Study:
- To assess the intrapatient variability of platelet δ-granule numbers using EM.
- To evaluate the reliability of EM for diagnosing δ-PSPD in young women with heavy menstrual bleeding.
Main Methods:
- Fifteen young women (11-17 years) with heavy menstrual bleeding underwent serial platelet EM testing over 3 months.
- Platelet aggregation tests were performed at the initial visit.
- Analysis focused on average δ-granules per platelet and consistency of results.
Main Results:
- Nearly half of the patients exhibited substantial variability in platelet EM results.
- Three patients initially had <2 average δ-granules, with two reverting to ≥2 on follow-up.
- Platelet aggregation was abnormal in 53% of patients, with no clear correlation to EM findings.
Conclusions:
- Significant intrapatient variability exists in platelet EM δ-granule counts.
- EM results for δ-PSPD can be inconsistent, even in serial testing.
- Providers should exercise caution using EM in isolation for δ-PSPD diagnosis.
Abstract:
Delta-granule platelet storage pool deficiency (δ-PSPD) is a qualitative platelet function defect associated with variable bleeding phenotypes. Platelet electron microscopy (EM) is commonly utilized to evaluate for δ-PSPD, but intrapatient variability in platelet δ-granule numbers by EM is currently unknown. Fifteen young women aged 11 to 17 years presenting to a young women's hematology clinic for the evaluation of heavy menstrual bleeding underwent platelet EM testing at their initial hematology clinic visit and at 1 and 3 months later. Platelet aggregation of platelet-rich plasma by light transmission was also performed on all patients at their initial visit. Eight patients had average δ-granules per platelet consistently ≥2. Three patients were found to have average δ-granules per platelet <2 on initial testing, 2 of which reverted to ≥2 on subsequent testing. When initial average δ-granules per platelet was ≥2, initial repeat testing remained so in 83% (95% confidence interval [CI], 52%-98%) of cases and subsequent repeat testing remained so in 75% (95% CI, 43%-95%) of the cases. Platelet aggregation testing was abnormal in 53% of patients, and there was no apparent correlation between platelet EM findings and platelet aggregation testing. In this small group of young women presenting for the evaluation of bleeding symptoms, we found that almost half of the patients had substantial variability in platelet EM results. Given other identified limitations in platelet EM testing, and the intrapatient variability identified in this study, providers should use caution in utilizing EM in isolation to diagnose δ-PSPD.
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