We thank Dr Kittisupamongkol for his thoughtful comments that were submitted in response to our article.1 Dr Kittisupamongkol correctly acknowledges that we did not rule out platelet-function disorders such as Glanzmann thrombasthenia. We wish to make a few points in response to these comments. First, we would like to make the case that our concern for Glanzmann thrombasthenia in this particular patient was appropriately low. Glanzmann thrombasthenia is an inherited deficiency in the integrin αIIbß3 platelet receptor, which results in impaired platelet adhesion.2 The incidence of this condition in the general population is exceedingly low, estimated to be 1 per 1 000 000 births, with an increased risk in areas where consanguineous marriages are common. Although patients may present with mild disease, Glanzmann thrombasthenia is generally considered a severe coagulation disorder, with most patients exhibiting signs such as menorrhagia, easy bruising, epistaxis, and excessive surgical bleeding early in life.3 Our patient exhibited no such signs and, furthermore, had undergone a major operation within the past 2 years without bleeding complications. Thus, it is extremely unlikely that Glanzmann thrombasthenia could account for the duodenal hematoma observed in this elderly gentleman. Second, we would like to reaffirm Dr Kittisupamongkol's comments by emphasizing that inherited platelet disorders, such as Glanzmann thrombasthenia or Bernard-Soulier disease, as well as other acquired defects, such as dense (δ) granule deficiency, are important for surgeons to be aware of, as these diseases can result in clinically significant bleeding (Mark D. Kligman, MD, unpublished data, August 2008). Dr Kittisupamongkol is also correct in his implication that many platelet disorders cannot be ruled out by a traditional coagulopathy workup in which only the protime, partial thromboplastin time, and platelet number (but not function) are examined. An elevated bleeding time is the classic sign of platelet disorders, as Dr Kittisupamongkol pointed out. This particular test, however, can be somewhat impractical to perform and is unnecessarily intrusive. Therefore, it is not performed on a routine basis at most hospitals in the United States (including ours). We instead prefer platelet-function analysis, platelet-rich plasma platelet–function analysis, and/or platelet-aggregation assays when clinically warranted to diagnose platelet disorders. Again, we thank Dr Kittisupamongkol for emphasizing the importance of inherited platelet disorders in the context of abnormal clinical bleeding. It is our opinion, however, that the clinical context in which our patient presented does not warrant further workup for rare congenital disorders of platelet function.
Weil BR, Howard TJ, Zyromski NJ. Is It Truly a Spontaneous Duodenal Hematoma?—Reply. Arch Surg. 2009;144(1):94. doi:10.1001/archsurg.2008.524
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