|Year : 2018 | Volume
| Issue : 1 | Page : 88-91
Glanzmann's thrombasthenia – A rare case report of spontaneous gingival bleeding
Vijayalakshmi Venkat, Sruti Kalluri, Satheesha Reddy Bandalore Hanumantha
Department of Oral Medicine and Radiology, AECS Maaruti College of Dental Sciences and Research Centre, Bengaluru, Karnataka, India
|Date of Submission||10-May-2017|
|Date of Acceptance||06-Apr-2018|
|Date of Web Publication||23-Apr-2018|
Dr. Vijayalakshmi Venkat
108, Hulimavu Tank Bund Road, Off Bannerghatta Road, BTM 6th Stage, 1st Phase, Kammanahalli, Near Meenakshi Temple, Bengaluru 560076, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Glanzmann's thrombasthenia is a rare congenital bleeding disorder which is associated with mutations in the genes encoding glycoproteins, gpIIb or gpIIIa resulting in platelet dysfunction. Here we present a case of 10 years old female patient with spontaneous bleeding gums since 4 days seeking dental treatment. She was under medical supervision following a diagnosis of some bleeding disorder before 4 years. With careful early diagnosis and proper supportive care Glanzmann's thrombasthenia has a very good prognosis.
Keywords: Blood platelets, hemorrhage, thrombasthenia
|How to cite this article:|
Venkat V, Kalluri S, Bandalore Hanumantha SR. Glanzmann's thrombasthenia – A rare case report of spontaneous gingival bleeding. J Indian Acad Oral Med Radiol 2018;30:88-91
|How to cite this URL:|
Venkat V, Kalluri S, Bandalore Hanumantha SR. Glanzmann's thrombasthenia – A rare case report of spontaneous gingival bleeding. J Indian Acad Oral Med Radiol [serial online] 2018 [cited 2021 Jul 26];30:88-91. Available from: https://www.jiaomr.in/text.asp?2018/30/1/88/230894
| Introduction|| |
Glanzmann's thrombasthenia was first documented in 1918 by Dr. Eduard Glanzmann, who described a functional abnormality of platelets with defective clot retraction. It is an autosomal recessive disorder where platelet count is normal or subnormal, bleeding time is prolonged, and there is deficiency or absence of platelet aggregation. Because of this deficiency of platelet function, it manifests as a bleeding disorder characterized by mucocutaneous hemorrhage of varying severity. The gene responsible is carried on the long-arm of chromosome 17 at q. The incidence is about 1 in 1,000,000 with an equal sex predilection. It has high incidence in populations where intermarriage is common.
| Case Report|| |
A female patient about 10 years of age reported to the department with a chief complaint of bleeding gums since 4 days. Her medical history showed multiple episodes of bleeding from the gums since 4 days which was spontaneous. There was also prolonged bleeding following minor trauma and hemetemesis. She also give a history of epistaxis and malena and also told that she had been diagnosed for some bleeding disorder. Immunization history was uneventful. She was born of non-consanguineous marriage. There was no significant family history of bleeding disorder. She also give history of taking tablet. She has been taking livogen and tranaxemic acid for the past 3 years, and three units of blood has been transfused till now. She was ectomorphic and poorly nourished. On examination, the child was conscious, cooperative, and was well-oriented. Pallor was noted in eyes and nailbeds of hands. Extraoral examination showed ecchymotic patches on both arms and wrist region [Figure 1]. Examination of liver and spleen showed no abnormality.
|Figure 1: Extraoral ecchymotic patches seen on right hand and wrist region|
Click here to view
Intraoral examination revealed spontaneous gingival bleeding from upper and lower tooth causing stains all over the surface [Figure 2]. Attached gingiva was firm and resilient in consistency. Stippling was present in attached gingiva and interdental papilla of upper and lower anterior tooth. Right [Figure 3] and left [Figure 4] buccal mucosa and soft palate showed generalized pallor. Dental caries with pulpal involvement was seen in 54. Numbers 54 and 83 showed grade II mobility. Considering the history and clinical examination, a provisional diagnosis of Glanzmann's thrombasthenia was given. Differential diagnoses considered were von Willebrand disease, Bernaud- Soulier syndrome, and platelet secretory defects.
|Figure 2: Spontaneous bleeding from the maxillary and mandibular anterior gingiva|
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The patient was then subjected to hematological investigation which showed normal platelet count of 1,78,000/mm 3, normal prothrombin time (11.2 s), normal partial thromboplastin time (28.5s), and normal thrombin time (14.9s), but bleeding time was prolonged >15 min. Factor VIII, fibrinogen, and von Willebrand factor showed normal values. Platelet aggregometry showed absence response to ADP, collagen, epinephrine, and arachidonic acid followed by disaggregation with normal dose ristocetin. Hemoglobin level was 12.1 g/dL, and mean corpuscular volume (MCV) was 75.6% which indicated microcytic hypochromic anemia.
An intraoral periapical radiograph was taken with respect to 54 which showed completely radiolucent crown structure showing grossly decayed tooth with physiologic resorption of the roots [Figure 5]. Based on the history, clinical findings, and investigations, final diagnosis of Glanzmann's thrombasthenia with microcytic hypochromic anemia was made.
Oral prophylaxis was done in the Department of Pedodontics and she was advised to take vitamin C supplements daily and to use soft tooth brush with fluoridated tooth paste. The treatment for grossly decayed 54 was delayed, taking into consideration the health condition of the child until hemorrhage had been controlled. She was referred to the general physician to obtain consent for the same. The recall schedule for this child was every 2 weeks initially which showed improvement in oral hygiene [Figure 6], followed by once in 3 months and then once in 6 months, stressing the importance of maintaining oral hygiene at all appointments.
| Discussion|| |
Glanzmann's thrombasthenia is a rare, autosomal recessive, bleeding disorder characterized by prolonged bleeding time, defective aggregation of platelets, and impaired clot retraction. George et al., divided Glanzmann's thrombasthenia in-to three groups as:
- Type- I: patients with less than 5% of GpIIb-IIIa
- Type- II: patients with 5%-20% of GpIIb-IIIa
- Type- III (variants): normal amounts of GpIIb-IIIa, but functionally inactive.
The common features of Glanzmann's thrombasthenia are bruising, epistaxis, gingival hemorrhage, and menorrhagia. Bruising typically occurs following minor trauma. The laboratory tests of these patients showed prolonged bleeding time, decreased or absence of clot retraction, and abnormal platelet aggregation responses to physiologic stimuli; all stood positive for this patient. The spontaneous bleeding episodes from the gingiva in the oral cavity and ecchymotic patches on the skin further confirmed the medical condition in this child.
Poor oral hygiene and local factors are the main etiological factors for bleeding gums. The others include systemic factors such as anemia, acute lymphocytic leukemia, uncontrolled hypertension, vitamin C and vitamin K deficiencies, and acute necrotizing ulcerative gingivitis. In the present case, the child's medical history revealed that she was a known case of Glanzmann's thrombasthenia since the age of 6 years. The differential diagnosis includes von Willebrand's disease, Bernard- Soulier syndrome, and platelet secretory defects.,
Normal ristocetin-induced platelet agglutination and normal platelet size clearly rule out Bernard-Soulier syndrome, a disorder of platelet adhesion. Inherited thrombocytopenias are eliminated by a normal platelet count. Normal coagulation parameters rule out clotting disorders that can also affect platelet function such as congenital afibrinogenemia and von Willebrand disease. Acquired thrombasthenia must be eliminated in the absence of a family history of the disease.
According to literature, a cure for the disease does not exist; the only effective therapy consists of transfusions of fresh platelets or platelet concentrates.
- Gelatin sponge or gauze, antifibrinolytic agents such as topical thrombin, and YAG laser can be used to control minor bleeding
- Desmopressin (DDAVP) has been tried in some patients with Glanzmann's thrombasthenia and may shorten bleeding time in patients with Type 2 only
- Oral contraceptives can regularize menstrual cycles and reduce bleeding
- Immunoabsorption is the removal of antibodies to platelets by plasma exchange with the use of protein-A sepharose columns which may transiently restore platelet efficacy. However, this technique is labor-intensive, and requires an adequate venous access. It is not of use in controlling active bleeding as this process requires several hours.
- Allogenic marrow transplant has been reported in two patients successfully with Glanzmann's thrombasthenia
- Tisseel is a fibrin sealant that acts both through its adhesive action and by direct contribution of fibrin to clot formation that prevents bleeding during extraction procedures 
- Tranexamic acid has also been used successfully in the form of a mouthwash or tablets after minor surgical procedures to inhibit postoperative bleeding episodes 
- Avoid prescribing medications that interfere in the normal function of platelets, such as aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs).
There have been no reports concerning the management of chronic gingival bleeding in children with Glanzmann's thrombasthenia. Spontaneous gingival hemorrhage has been minimized through maintenance of good oral hygiene. The role of the dentist begins with preventive therapy, and educating the patient to maintain a good oral hygiene and to prevent inadvertent bleeding from the oral cavity after dental treatment. Platelet transfusion is essential before any dental procedure to reduce the risk of hemorrhage.
Under home care measures, the child was advised to consume only foods of soft texture with limited temperature variations to avoid any bleeding episodes. Wet gauze cleansing was recommended to maintain oral hygiene. The oral health of these children is usually neglected because of the severity of the medical condition. But bleeding occurring because of gingival inflammation or infection (as in this child) can be life-threatening. Hence, strict oral hygiene measures such as brushing twice daily with a soft tooth brush and fluoridated toothpaste with parental supervision, diet counseling sessions, and regular dental checkups were advocated and repeatedly reminded to the parent and the child alike.
| Conclusion|| |
Although Glanzmann'thrombasthenia is one of the rare bleeding disorders, one should be aware of this condition while evaluating patients with spontaneous gingival bleeding in the setting of normal platelet count. Early diagnosis and prompt treatment carry good prognosis. Communities should be counseled to avoid consanguineous marriage and related hazards. Patient should be educated for life-style modification and regarding measures to avoid bleeding episodes which includes avoidance of anti-platelet drugs, avoid trauma, and maintain good oral hygiene.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]