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MISCELLANEOUS
Year : 2007  |  Volume : 25  |  Issue : 5  |  Page : 37-40
 

Glanzmann's thrombasthenia associated with HBsAg-positive child: A case report


Department of Oral Medicine and Radiology, Bapuji Dental College and Hospital, Davanagere, Karnataka, India

Correspondence Address:
G Poornima
Department of Oral Medicine and Radiology, Bapuji Dental College and Hospital, Davanagere - 577 004, Karnataka
India
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Source of Support: None, Conflict of Interest: None


PMID: 17921640

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   Abstract 

Glanzmann's thrombasthenia is a rare hemorrhagic disorder characterized by prolonged bleeding time and diminished clot retraction. The disease is marked by frequent mucocutaneous hemorrhage which is mainly due to qualitative defects of platelets. A case of a 14-year-old HBsAg-positive adolescent male with Glanzmann's thrombasthenia has been presented.


Keywords: Glycoproteins, platelet disorder, thrombasthenia


How to cite this article:
Poornima G, Shivaprasad S, Ashok L. Glanzmann's thrombasthenia associated with HBsAg-positive child: A case report. J Indian Soc Pedod Prev Dent 2007;25, Suppl S1:37-40

How to cite this URL:
Poornima G, Shivaprasad S, Ashok L. Glanzmann's thrombasthenia associated with HBsAg-positive child: A case report. J Indian Soc Pedod Prev Dent [serial online] 2007 [cited 2019 Dec 6];25, Suppl S1:37-40. Available from: http://www.jisppd.com/text.asp?2007/25/5/37/34746



   Introduction Top


Glanzmann's thrombasthenia is a rare hemorrhagic autosomal recessive bleeding disorder characterized by prolonged bleeding time and diminished clot retraction. The disease is marked by frequent mucocutaneous hemorrhages either due to defective function of the platelets or lack of fibrinogen-binding membrane receptor glycoproteins IIb/IIIa which are located on the surface of the platelets. [1],[2]


   Case Report Top


A 14-year-old adolescent male patient reported to our department with chief complaint of irregularly placed lower front teeth. Patient noticed crowding at the age of 7 years after the eruption of lower permanent incisors without exfoliation of deciduous incisors. Patient had difficulty in brushing his teeth and wanted to get the irregularly erupted teeth corrected, with prime concern for esthetics.

His past medical history revealed that he had history of epistaxis, since 2 months after birth; associated recurrent fever; and bleeding from ears. At the age of 4 months, the patient was diagnosed as having Glanzmann's thrombasthenia. He also had history of easy bruising, epistaxis, bloody stools and bleeding gums.

The patient was diagnosed as HBsAg positive at the age of 5 years, probably due to repeated blood transfusions.

Past laboratory investigations revealed:

  • Normal platelet count
  • Increased bleeding time
  • Clotting time was 4.15 minutes
  • Normal coagulation parameters
  • Poor clot retraction time (20%)
  • No response to adenosine diphosphate (ADP) and epinephrine on platelet aggregometer study.


His family history revealed that there was no history of consanguineous marriage, and no other family member had similar problems.

General physical examination revealed that the patient was of moderate built; had no signs of anemia, cyanosis; and all his vital signs were within normal limits.

Multiple petechiae were present on the face [Figure - 1], neck, right and left upper and lower limbs [Figure - 2],[Figure - 3], chest, abdomen and back. Right and left submandibular lymph nodes were palpable; there was one palpable lymph mode measuring approximately 1 1 cm, mobile, firm in consistency and tender on palpation, on each side.

Intraoral examination

Petechiae were present on the left buccal mucosa in the line of occlusion in relation to 27 and 37 [Figure - 4]. There was spontaneous bleeding from the sulcus in relation to 34 and 35 [Figure - 5]. Hard tissue examination revealed a complete set of permanent dentition; and over retained 72, 73, 82, 83 and 32, 33, 42, 43 were lingually erupting. The patient was subjected to radiographic investigation. The orthopantomograph revealed complete set of dentition with retained 72, 73, 82, 83 and erupting 32, 33, 42, 43 [Figure - 6]. There was no other radiographic change.

Management

Bleeding from the sulcus was controlled by local hemostatic agent i.e., inj streptochrome, and the patient was referred to a physician for evaluation.


   Discussion Top


Glanzmann's thrombasthenia is an inherited autosomal recessive disorder of platelet function, which was described first in 1918 by Dr. Glanzmann, a German pediatrician. [3],[4]

This disease begins in infancy and is usually diagnosed in early childhood. The severity of disease tends to decrease with age. [2]

Etiology and pathogenesis

Although the number of circulating platelets is normal in Glanzmann's thrombasthenia, the aggregation capacity of the platelets is defective, possibly due to a qualitative and/or a quantitative defect in GPIIb/IIIa glycoprotein complex found on the platelet membrane. [5],[6],[7]

Genetic aspects

Glanzmann's thrombasthenia is seen in populations with consanguinity. The genes for GPIIb and GPIIIa are both located on the long arm of chromosome 17 at q 21-23 . [5]

Classification

Caen in the year 1972 proposed the following classification (before the GPIIb-IIIa abnormality was discovered) on the basis of platelet fibrinogen content and clot retraction:

Type I thrombasthenia characterized by a lack of platelet aggregation, an apparent absence of platelet fibrinogen and a profound defect in clot retraction

Type II subgroup characterized by a lack of aggregation accompanied by subnormal but clearly detectable levels of platelet fibrinogen and only a moderately defective clot retraction [2]

George et al. [8] divided the patients into three groups based on the platelet fibrinogen content and clot retraction:

Type I - patients having less than 5% GPIIb-IIIa

Type II - patients having 5-20% of normal amount of GPIIb-IIIa

Variants - patients having 'half the normal' to normal amounts of GPIIb-IIIa but abnormal receptor function. [2],[8]

Clinical features

Purpura usually begins in infancy and is unremitting. Bleeding usually begins early in childhood and follows a characteristic pattern of recurrent, spontaneous episodes of mucosal and cutaneous hemorrhage, epistaxis, frequent superficial bruises, gingival bleeding, menorrhagia, prolonged bleeding from superficial injuries, surgical procedures, dental extractions and during postpartum. [3],[8],[9],[10] In our case also, there was epistaxis, easy bruising, bloody stools, gingival bleeding and extensive petechiae.

Differential diagnosis

In patients with mucocutaneous bleeding, prolonged bleeding time and normal platelet count, differential diagnosis includes Von Willebrand's disease, Bernard Soulier syndrome and platelet secretory defects, in addition to Glanzmann's thrombasthenia. [2]

Laboratory investigations

The hematologic factors include a normal platelet count with prolonged bleeding time, a defective clot retraction and failure of platelets to aggregate in response to ADP, epinephrine, collagen, thrombin or archidonic acid, 5-hydroxytryptamine; but aggregation occurs normally with ristocetin and bovine factor VIII. [5, 8, 9] Coagulation tests such as prothrombin time and partial thromboplastin time are normal; clot retraction in the presence of thrombasthenic platelets is either absent or reduced. [5]

Management

A cure for the disease does not exist; the only effective therapy consists of transfusions of fresh platelets or platelet concentrates. [10]

Dental management

Local bleeding can usually be controlled by local measures - e.g., epistaxis and gingival bleeding are successfully controlled in most patients by conventional conservative measures with application of gel foam soaked in thrombin or nasal packing. Regular dental care to carefully clean the teeth and for removal of plaque is essential to prevent gingival bleeding. Local anesthesia for dental procedures with 2% lidocaine with 1:100,000 epinephrine can be given without risk of bleeding. Systemic Epsilon amino caproic acid administration can be used as an adjunct to platelet transfusion in patients with thrombasthenia. A dose of 40 mg/kg four times daily is recommended. [6]

Oral contraceptives have been useful in controlling menorrhagia; fibrinolytic inhibitors such as E-aminocaproic acid, in addition to platelet transfusion, may be useful in controlling bleeding after dental extractions. [5] Some investigators recommend the use of local hemostatics (microfibrillar bovine collagen, Avitene, cellulose, oxycel) for minor surgical interventions, such as periodontal prophylaxis. In case of major interventions, these drugs combined with a platelet concentrate transfusion can be administered if necessary. [1],[3],[9]

Patients affected with Glanzmann's thrombasthenia are at risk due to:

  • Hemorrhagic complications, which may be overcome by supplemental therapy
  • Development of autoantibodies following a transfusion of platelets and post-transfusion thrombocytopenic purpura. [10]



   Conclusion Top


Although Glanzmann's thrombasthenia is one of the rare bleeding disorders, one should be aware of this condition while evaluating patients with spontaneous gingival bleeding; hence the role of the dentist lies in giving proper periodontal care and encouraging maintenance of optimal oral hygiene, which is essential for both local and systemic health.

 
   References Top

1.Kantarci A, Cebeci I, Firatli E, Atamer T, Tuncer O: Periodontal management of Glanzmann's thrombasthenia: Report of 3 cases. J Periodontal 1996;67:816-20  Back to cited text no. 1    
2.Bisch FC, Bowen KJ, Hanson BS, Kudryk VL, Billman MA. Dental consideration for a Glanzmann's thrombasthenia patient. J Periodontal 1996;67:536-40  Back to cited text no. 2    
3.Fernandes Gomes M, de Melo RM, Plens G, Pontes EM, Silva MM, da Rocha JC, et al . Surgical and clinical management of a patient with Glanzmann's thrombasthenia: A case report. Quintessence Int 2004;35:617-20  Back to cited text no. 3    
4.Tullu MS, Dixit PS, Nair SB, Kamat JR, Vaswani RK, Shetty SD, et al . Glanzmann's thrombasthenia. Indian J Pediatr 2001;68:563-6  Back to cited text no. 4    
5.Lee R, Forester J, Lukens J, Paraskevas F, Greer JP, Rodgers GM. Wintrobe's clinical hematology. 10 th ed. vol-2. Williams and Wilkins A Waverly Company: p. 16  Back to cited text no. 5    
6.Rakocz M, Lavie G, Martinowitz U. Glanzmann's thrombasthenia: The use of autololous fibrin glue in tooth extractions. ASDC J Dent Child 1995;62:129-31  Back to cited text no. 6    
7.Hoffman R, Benz EJ, Shattil SJ, Furie B, Cohon HJ, Silberstein LE. Hematology basic principles and practice. 2 nd ed. Churchill Livingstone Inc: 1995. p. 1913  Back to cited text no. 7    
8.George JN, Caen JP, Nurden AT. Glanzmann's thrombasthenia: The spectrum of clinical disease. Blood 1990;75:1383-95  Back to cited text no. 8    
9.Jasmin JR, Dupont D, Velin P. Multiple dental extractions in a child with Glanzmann's thrombasthenia: Report of a case. ASDC J Dent Child 1987;54:208-10  Back to cited text no. 9    
10.Macintyre DE, Gordon JI. Platelets in biology and pathology. Elsevier Amsteran; New York, Oxford; 1987. p. 69-86  Back to cited text no. 10    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]



 

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    Abstract
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    Case Report
    Discussion
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    References
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