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

Author:

Kouno Hiroyoshi1ORCID,Kunishima Shinji2ORCID,Takebe Junko3,Uchiyama Toru3ORCID,Kaname Tadashi4ORCID,Ishiguro Akira5ORCID

Affiliation:

1. Hiroshima City Hiroshima Citizens Hospital Hiroshima Japan

2. Gifu University of Medical Science Gifu Japan

3. Department of Human Genetics National Center for Child Health and Development (NCCHD) Tokyo Japan

4. Department of Genome Medicine, National Center for Child Health and Development (NCCHD) Tokyo Japan

5. Center for Postgraduate Education and Training and Division of Hematology, National Center for Child Health and Development (NCCHD) Tokyo Japan

Abstract

AbstractIntroductionAlthough 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.MethodsWe 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.ResultsMedian 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%.ConclusionOur study strongly suggests that IPF% is useful in the differential diagnosis between MYH9 disorders and other types of thrombocytopenia.

Funder

Japan Agency for Medical Research and Development

National Center for Child Health and Development

Publisher

Wiley

Subject

Biochemistry (medical),Clinical Biochemistry,Hematology,General Medicine

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