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SynopsisCodesBest TestsReferences
May-Hegglin anomaly
Other Resources UpToDate PubMed

May-Hegglin anomaly

Other Resources UpToDate PubMed

Synopsis

A rare autosomal dominant genetic disorder characterized by giant platelets, thrombocytopenia, and leukocytes with Dohle body inclusions. Caused by abnormalities in gene MYH9. Signs and symptoms include prolonged bleeding time, purpura, epistaxis, gingival hemorrhage, intracranial bleeding, menorrhagia, and easy bruising. Patients can be asymptomatic, or symptoms may present at birth. Males and females are equally affected. Treatment is only required during episodes of substantial bleeding. Precautions in case of excess bleeding should be taken with pregnant patients and during surgery or dental work. Closely related to Sebastian syndrome, Fechtner syndrome, and Epstein syndrome.

Codes

ICD10CM:
D72.0 – Genetic anomalies of leukocytes

SNOMEDCT:
80217004 – May-Hegglin inclusion

Best Tests

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References

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Last Updated:01/19/2022
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May-Hegglin anomaly
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A medical illustration showing key findings of May-Hegglin anomaly : Bleeding time prolonged, Easy bruising, Ecchymosis, Menorrhagia, Gingival bleeding, PLT decreased, Recurrent epistaxis
Copyright © 2024 VisualDx®. All rights reserved.