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Brent R. Weil, MD; Thomas J. Howard, MD; Nicholas J. Zyromski, MD

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In reply

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 IIbß3 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 . . . [Full Text of this Article]

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