May-Hegglin Anomaly Follow-up

  • Author: Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Mar 29, 2012
 

Complications

Most individuals with May-Hegglin anomaly (MHA) do not have bleeding problems; however, severe bleeding can occur.[10] The bleeding risk is increased by taking drugs that decrease platelet function.

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Prognosis

Most patients with May-Hegglin anomaly do not have clinically significant problems with bleeding and, therefore, do not require treatment.

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Patient Education

Individuals with May-Hegglin anomaly should understand their personal risk of bleeding. They should understand that their bleeding risks are associated with the degree of thrombocytopenia.

Before patients undergo surgical procedures or in patients with trauma, the diagnosis of May-Hegglin anomaly must be discussed because special precautions and procedures may be required to prevent bleeding complications.

Individuals with May-Hegglin anomaly should be educated to avoid drugs (eg, aspirin) that can adversely affect platelet function.

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Contributor Information and Disclosures
Author

Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP  Associate Professor of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute

Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Royal College of Physicians of the United Kingdom

Disclosure: Nothing to disclose.

Specialty Editor Board

Gary R Jones, MD  Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Gary D Crouch, MD  Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Frank E. Shafer, MD, to the development and writing of this article.

References

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Blood smear (original magnification X2000) in a patient with May-Hegglin anomaly (MHA) demonstrates a characteristic giant platelet with poorly defined granulation. A normal-sized platelet is also present. The trilobed neutrophil contains a large, well-defined, basophilic, peripherally placed cytoplasmic inclusion body (resembling a Döhle body). Used with permission from Little, Brown.
Table. Clinical Features of MYH9 -Related Thrombocytopenias[15]
ConditionMacrothrombocytopeniaGranulocyte inclusionsNephritis and DeafnessCataracts
MHAYesLinear DöhlelikeNoNo
Epstein syndromeYesAbsent or faintYesNo
Fechtner syndromeYesSpherical granulesYesYes
Sebastian syndromeYesSpherical



granules



NoNo
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