ABSTRACT
Hermansky-Pudlak Syndrome (HPS) is a rareautosomal recessive disorder characterized byOculocutaneous Albinism (OCA), platelet disorder,and ceroid accumulation. It is common in North WestPuerto Rico region, and the incidence reported is1/500000. It is a rare genetic disorder with plateletdysfunction resulting in bleeding diathesis. Here wereport one such rare case of HPS type 2 in a 7-year-oldboy with difficulty in chewing.
ABSTRACT
Patients with platelet-mediated disorders often present clinical manifestations of bruising and bleeding. Although these changes are detected most frequently on the skin, the oral cavity also may exhibit signs of bleeding. In this report, we describe a patient who presented isolated oral features of hemorrhagic bullae with bleeding, indicative of a bleeding disorder. Results of laboratory tests revealed severe thrombocytopenia and a careful history disclosed dengue fever as the cause. The importance of recognizing the oral manifestations of thrombocytopenia is highlighted here, since the oral cavity is a frequent site of hemorrhage and could be the only manifestation of the disease.
Subject(s)
Humans , Female , Dengue/diagnosis , Mouth Diseases/diagnosis , Blood Platelets/pathology , Thrombocytopenia/diagnosis , Thrombocytopenia/pathologyABSTRACT
Glanzmann's thrombasthenia is a qualitative platelet disorder characterized by a deficiency in the platelet membrane glycoproteins IIb/IIIa. It belongs to a group of hereditary platelet disorders typified by normal platelet numbers and a prolonged bleeding time. The severity of bleeding does not correlate with the severity of the platelet glycoprotein IIb/IIIa abnormality. The present case report describes the periodontal treatment of a patient with Glanzmann's thrombasthenia. A 30-year-old female with a history of Glanzmann's thrombasthenia was referred for gingival bleeding on tooth brushing and discomforts in #38 area. The periodontal finding revealed a diagnosis of localized slight adult periodontitis. Root planing and extraction of #38 was performed under 12 pack of platelets transfusion and digital compression was done for hemostasis. The gingival bleeding ceased within a day in maxilla and 2 days later in mandible. 42 pack of platelets was administered for 3 days of post-treatment and for iron-deficiency anemia 3 pack of RBCs was transfused 2 days later. 1 week later the inflammation in gingiva disappeared and gingival stippling appeared. The clinical result we got was good and in such a medically compromised patient it is an ability to maintain a proper oral hygiene that is essential both for oral and systemic health.