May-Hegglin Anomaly Medication

Updated: Feb 28, 2019
  • Author: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP; Chief Editor: Hassan M Yaish, MD  more...
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Medication

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.

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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).

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