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May-Hegglin Anomaly Medication

  • Author: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP; Chief Editor: Hassan M Yaish, MD  more...
Updated: May 03, 2016

Medication Summary

Most patients with May-Hegglin anomaly (MHA) do not have clinically significant problems with bleeding and do not require treatment. Corticosteroids and splenectomy are ineffective. On rare occasions when patients have severe bleeding, platelet transfusions may be required. Prophylactic platelet transfusions are not routinely used before surgery and delivery. Intravenous desmopressin has been used preoperatively as a nonspecific agent to improve hemostasis. Stimate (nasal desmopressin) should be as effective in such cases.


Pituitary Hormone

Class Summary

Desmopressin acetate, a synthetic analogue of vasopressin, may be used before surgery.

Desmopressin acetate (DDAVP, Stimate)


Desmopressin acetate releases von Willebrand protein from endothelial cells. It improves bleeding time and hemostasis in patients with some von Willebrand factor (ie, with mild and moderate von Willebrand disease without abnormal molecular forms of von Willebrand protein). It is effective in uremic bleeding. Tachyphylaxis usually develops after 48 hours, but desmopressin acetate can be effective again after several days. A nasal solution is available in strengths of 0.1 mg/mL (10 µg/0.1 mL) and 0.6 mg/mL (1.5 mg/2.5 mL).

Contributor Information and Disclosures

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP Professor of Pediatrics, Albany Medical College; Chief, Division of Pediatric Hematology-Oncology, John and Anna Landis Endowed Chair for Pediatric Hematology-Oncology, Medical Director, Melodies Center for Childhood Cancer and Blood Disorders, Albany Medical Center

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, International Society of Pediatric Oncology

Disclosure: Nothing to disclose.

Chief Editor

Hassan M Yaish, MD Medical Director, Intermountain Hemophilia and Thrombophilia Treatment Center; Professor of Pediatrics, University of Utah School of Medicine; Director of Hematology, Pediatric Hematologist/Oncologist, Department of Pediatrics, Primary Children's Medical Center

Hassan M Yaish, MD is a member of the following medical societies: American Academy of Pediatrics, New York Academy of Sciences, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Michigan State Medical Society

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.

Frank E Shafer, MD Associate Professor, Department of Pediatrics, Section of Hematology-Oncology, St Christopher's Hospital for Children, MCP Hahnemann University School of Medicine

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.

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Blood smear (original magnification ×2000) in patient with May-Hegglin anomaly (MHA) demonstrates characteristic giant platelet with poorly defined granulation. Normal-sized platelet is also present. Trilobed neutrophil contains large, well-defined, basophilic, peripherally placed cytoplasmic inclusion body (resembling Döhle body). Image used with permission from Little, Brown.
Table. Clinical Features of MYH9 -Related Thrombocytopenias
Condition Macrothrombocytopenia Granulocyte inclusions Nephritis and Deafness Cataracts
MHAYesLinear DöhlelikeNoNo
Epstein syndromeYesAbsent or faintYesNo
Fechtner syndromeYesSpherical granulesYesYes
Sebastian syndromeYesSpherical granulesNoNo
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