May-Hegglin Anomaly Treatment & Management

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

Approach Considerations

The literature is conflicting, but most patients with May-Hegglin anomaly (MHA) do not appear to have clinically significant bleeding problems, and specific treatment is not required. Corticosteroids and splenectomy are ineffective. In rare patients with severe bleeding, platelet transfusion may be required.

Patients with MHA who undergo normal vaginal or cesarean delivery do not appear to have a significantly increased risk of bleeding. [23, 24]

For patients with MHA scheduled for surgery, a careful personal and family history of bleeding tendency should be obtained and a manual platelet count performed to determine the actual risk for bleeding. Intravenous desmopressin acetate (DDAVP) may be valuable. [19, 25] A patient with MHA who successfully underwent craniotomy after DDAVP infusion alone has been described. [19] Routine prophylactic platelet transfusions are not usually indicated, though it is prudent to ensure that platelets are available in case unexpected bleeding occurs.

Depending on the degree of thrombocytopenia and family history, individuals may be at an increased risk for bleeding, and refraining from participation in contact or collision sports may be prudent.

A hematologist should be consulted to assist in the management of patients who are undergoing surgery or vaginal delivery and patients who have experienced severe trauma.