{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T02:52:59Z","timestamp":1780541579763,"version":"3.54.1"},"reference-count":23,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2007,7,26]],"date-time":"2007-07-26T00:00:00Z","timestamp":1185408000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2007,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Objective:\u2002<\/jats:bold> Hereditary thrombocytopenias characterized by mutations in the gene for non\u2010muscle myosin heavy chain IIA (NMMHC\u2010IIA) are known as MYH9\u2010related hereditary macrothrombocytopenia, and include the May\u2013Hegglin anomaly, Sebastian platelet syndrome, Fechtner syndrome, and Epstein syndrome. Despite the presence of thrombocytopenia, these patients often have only mild or non\u2010bleeding phenotypes. A major risk for these patients can be inappropriate treatment with long\u2010term corticosteroids or splenectomy for misdiagnosed chronic autoimmune thrombocytopenia, as well as inadequate peri\u2010 and postoperative management.<\/jats:p><jats:p><jats:bold>Methods:\u2002<\/jats:bold> Using the case of a 44\u2010yr\u2010old male with Fechtner syndrome (macrothrombocytopenia, leukocyte inclusions, sensorineural deafness, glomerulonephritis) who underwent neurosurgery for an intracerebral arteriovenous malformation, we describe current methods to diagnose hereditary MYH9\u2010related macrothombocytopenia by analysis of the blood smear, immunofluorescence staining of the NMMHC\u2010IIA in leucocytes, and by MYH9\u2010gene sequencing.<\/jats:p><jats:p><jats:bold>Results:\u2002<\/jats:bold> Clusters of NMMHC\u2010IIA in granulocytes and a R1165C mutation in the MYH9\u2010gene in two macrothrombocytopenic family members confirmed the diagnosis of a MYH9\u2010related disease. The patient had no bleeding diathesis by history or physical examination. Thus no perioperative prohemostatic pharmacologic therapies or transfusions were given, with only minimal bleeding observed. Postoperative antithrombotic thromboprophylaxis was not given because of anticipated enhanced risk for bleeding. However, the patient developed symptomatic pulmonary embolism on postoperative day 6, which was successfully managed with 8\u2003months of anticoagulation.<\/jats:p><jats:p><jats:bold>Conclusion:\u2002<\/jats:bold> MYH9\u2010related hereditary macrothrombocytopenia does not necessarily protect against postoperative venous thromboembolism, and affected patients who do not evince bleeding diathesis should be considered for routine postoperative pharmacologic thromboprophylaxis.<\/jats:p>","DOI":"10.1111\/j.1600-0609.2007.00913.x","type":"journal-article","created":{"date-parts":[[2007,7,27]],"date-time":"2007-07-27T13:12:51Z","timestamp":1185541971000},"page":"263-268","source":"Crossref","is-referenced-by-count":15,"title":["Perioperative management of MYH9 hereditary macrothrombocytopenia (Fechtner syndrome)"],"prefix":"10.1111","volume":"79","author":[{"given":"Kathleen","family":"Selleng","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lena E.","family":"Lubenow","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Greinacher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Theodore 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