Abstract
Congenital thrombocytopenia is a rare, intractable hereditary disease characterized by thrombocytopenia and abnormalities of platelet function. In contrast, idiopathic thrombocytopenic purpura (ITP) is a more frequent thrombocytopenic disease. Furthermore, it is possible that chronic and refractory ITP include congenital thrombocytopenia. We describe a case in which we extracted a tooth and performed hemostasis in an 87-year-old man with a suspected congenital X-linked thrombocytopenia (XLT)-related severe bleeding tendency. XLT is very rare in congenital thrombocytopenia. The patient had a platelet count of 2.0×104/μL and received 20 units of platelet concentrate before and after the extraction in liaison with our hematology and oncology department. We have previously reported a model on hemostasis management and extraction of Bernard–Soulier Syndrome (BSS) patients with congenital thrombocytopenia. The extraction sites were packed with gelatin sponges, and sutured, then a splint was used for hemostasis. Excellent hemostasis was achieved with minimal required platelet infusion.
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