Measurement of immature platelet fraction is useful in the differential diagnosis of MYH9 disorders

Introduction: Although the presence of large and giant platelets is important in screening for MYH9 disorders, platelet morphology evaluation is dependent on operator subjectivity. Immature platelet fraction (IPF%) is widely used in clinical practice because of its rapidity and reproducibility; however, IPF% has been rarely analyzed in MYH9 disorders. Therefore, our study aimed to clarify the usefulness of IPF% in the differential diagnosis of MYH9 disorders.

Methods: We assessed 24 patients with MYH9 disorders, 10 with chronic immune thrombocytopenia (cITP), 14 with myelodysplastic syndromes (MDS) with thrombocytopenia (<100 × 10⁹ /L), and 20 healthy volunteers. Platelet-related data, including IPF% and platelet morphology (diameter, surface area, and staining), were retrospectively analyzed.

Results: Median IPF% in MYH9 disorders, 48.7%, was significantly higher than in all other groups (cITP: 13.4%, MDS: 9.4%, controls: 2.6%). IPF% in MYH9 disorders was significantly negatively correlated with platelet count and significantly positively correlated with the diameter and surface area of platelets, but a correlation was not found between IPF% and platelet staining. The area under the curve of IPF% for the differential diagnosis of MYH9 disorders was 0.987 (95% CI: 0.969-1.000), with a sensitivity of 95.8% and specificity of 93.2% when the cutoff value of IPF% was 24.3%.

Conclusion: Our study strongly suggests that IPF% is useful in the differential diagnosis between MYH9 disorders and other types of thrombocytopenia.