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CASE REPORT
Year : 2014  |  Volume : 32  |  Issue : 2  |  Page : 181-184
 

Hematological and surgical management in Glanzmann's thrombasthenia: A case report


1 Department of Pediatric Dentistry, Amrita School of Dentistry, Kochi, Kerala, India
2 Department of Oral and Maxillofacial Surgery, Amrita School of Dentistry, Kochi, Kerala, India
3 Department of Craniofacial Aesthetics, V. G. Saraf Hospital, Ravipuram, Kochi, Kerala, India

Date of Web Publication17-Apr-2014

Correspondence Address:
Anjana Gopalakrishnan
Opposite Akshaya Hospital, Kadavanthra, Kochi, Kerala - 682 020
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.131000

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   Abstract 

Glanzmann's thrombasthenia (GT) is a rare, congenital, and moderate to severe platelet disorder. The bleeding time is increased, due to lack of platelet aggregation, since the patients with GT have deficient or dysfunctional integrin membrane glycoproteins IIb and IIIa essential for platelet aggregation. Children with GT are mostly diagnosed very early in life due to the spontaneous and unexplained mucocutaneous bleeding. It is quite a challenging task when any surgery is indicated for children with GT. This case report is about the medical and surgical management of an 11-year-old girl diagnosed with Glannzmann's thrombasthenia who had to undergo a maxillary cyst enucleation.


Keywords: Cyst enucleation, Glanzmann′s thrombasthenia, platelet disorder


How to cite this article:
Gopalakrishnan A, Veeraraghavan R, Panicker P. Hematological and surgical management in Glanzmann's thrombasthenia: A case report. J Indian Soc Pedod Prev Dent 2014;32:181-4

How to cite this URL:
Gopalakrishnan A, Veeraraghavan R, Panicker P. Hematological and surgical management in Glanzmann's thrombasthenia: A case report. J Indian Soc Pedod Prev Dent [serial online] 2014 [cited 2019 Jul 16];32:181-4. Available from: http://www.jisppd.com/text.asp?2014/32/2/181/131000



   Introduction Top


Glanzmann's thrombasthenia (GT) is a very rare, congenital, bleeding diathesis first described by Dr Eduard Glanzmann in 1918, who identified this disease in children from a village high in Swiss Alps where marriages between close relatives were in practice. It affects about one in every million people with only 130 cases reported till 1969. [1],[2] It is an autosomal recessive platelet disorder, prevalent in populations where consanguineous marriages are common. [3],[4] It affects males and females equally since the gene responsible for GT is carried on the long arm of chromosome 17(17; q 21-22) one of the 22 pairs of autosomal chromosomes, in humans. [1],[3] It is characterized by prolonged bleeding time, while platelet count, and morphology as well as prothrombin time (PT) and partial thromboplastin time (PTT) remains unaffected. [3],[4] GT is caused by the qualitative and quantitative defects of the platelet membrane glycoproteins IIb-IIIa (α IIbβ3 ) complex which are fibrinogen receptors required for fibrinogen dependent platelet-to-platelet aggregation. [2],[5],[6]

Clinical features

Mucocuteanous bleeding, gingival hemorrhages, menorrhagia, gastrointestinal bleeding, hematuria, muscle hemangioma, and hemarthroses can be seen in patients with GT. Brain hemorrhage is rare. Prolonged bleeding can occur from dental extractions, superficial injuries, surgeries, and during postpartum. Very good prognosis is seen with supportive care and as the age increases, the severity of bleeding decreases. [1],[2],[3],[4],[5]


   Case Report Top


An 11-year-old girl reported with hard swelling on the right maxillary arch noticed since a month which was enlarging [Figure 1]. She was the first daughter of a nonconsanguineous marriage, but parents were from a community where consanguineous marriages were common. They gave no family history of any bleeding disorder. She had history of purpuric patches since birth, prolonged bleeding after vaccination, and primary tooth extraction, gingival, and mucocutaneous bleeding [Figure 2] and an episode of gastrointestinal bleed in 2007 and was diagnosed to have GT. Past hematological evaluation reports showed prolonged bleeding time, decreased clot retraction, and abnormal platelet aggregation response to physiologic stimuli. Platelet counts and morphology were normal. Her immunization schedule was complete and she had achieved all the milestones of growth at the correct time.
Figure 1: Extraoral swelling

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Figure 2: Hemorrhage from mucous membrane

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Intraoral examination revealed a bony swelling on the buccal side of carious 54 and 55 [Figure 3]. A smaller swelling was appreciated on the palate as well. Orthopantomogram (OPG) showed the presence of a cyst-like lesion involving the crowns of unerupted permanent canine and premolars as well as the roots of 54 and 55. A three-dimensional OPG was made to assess the exact extent of the lesion and to be minimally invasive, so as to reduce the amount of trauma caused by the surgical intervention [Figure 4]. The lesion was revealed to be more towards the palate, and the buccal swelling was caused by the displaced premolars. It was decided to do a cyst enucleation and extraction of carious primary teeth. Patient was admitted and sent for relevant blood investigations, serology, and preanesthetic check. Since the hemoglobin count was low, she was given ferrous fumarate. Preoperatively, she was also given tranexamic acid 500 mg thrice daily for 2 days, followed by transfusion of seven units of single donor platelets and 1 unit packed red blood cells (PRBCs). She was taken up for surgery on the 3 rd day of admission. Root stumps of primary molars were extracted, and a palatal mucoperiosteal flap was raised up to the midline. The cystic lining was enucleated out leaving the permanent canine and premolars undisturbed [Figure 5]. Surgicel (oxidized cellulose polymer-polyanhydroglucuronic acid) was placed and the wound was sutured up. Postsurgically, 3 units of single donor platelets were transfused. No significant bleeding was noticed during the hospital stay. Tramadol was administered for pain control as nonsteroidal anti-inflammatory drugs (NSAIDs) could facilitate bleeding. Tranexamic acid oral swish (50%) was given thrice daily. She was discharged after 3 days with a good hemoglobin count of 10.9 g% and was advised to come for review after a week with the complete blood count (CBC) report. Histopathological evaluation confirmed the diagnosis as radicular cyst [Figure 6]. One week later, there was a delayed severe bleed from the surgical site and a syncopal attack at home. Two units of pooled platelets were transfused at the local hospital and she was referred back to our center. She had another syncopal attack at the casualty and was on the verge of hypovolemic shock. Uncrossmatched blood transfusion had to be performed and after stabilization, she was admitted in the Medical Intensive Care Unit (MICU). One unit PRBC and one pint pooled platelets were transfused. The poorly organized clot was removed and pressure pack was given to stop the bleeding. She was observed for 2 more days before discharge. There were no further bleeding episodes. A 5-month follow-up was done, at the end of which there was considerable reduction in the size of the lesion when evaluated with a three-dimensional OPG and the permanent premolars had erupted [Figure 7] and [Figure 8].
Figure 3: Swelling seen on the buccal aspect of 54 and 55

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Figure 4: Three-dimensional radiographic images of the lesion

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Figure 5: Cyst enucleation

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Figure 6: Histopathological image

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Figure 7: Five months postoperative clinical appearance

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Figure 8: Three-dimensional radiographic images after 5 months

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   Discussion Top


Pediatric dentists come across many intriguing medical conditions when children are brought for dental management. Excessive bleeding after extractions or shedding of primary teeth, unexplained and spontaneous bleeding from gingiva, and mucous membranes or petechiae, purpura or echymosis on the skin or mucous membrane should alert us on the possibility of a bleeding diathesis. Apart from GT, the conditions which show prolonged bleeding time, normal platelet count and morphology, and mucocutaneous bleeding include Von Willebrands disease, Bernard-Soulier syndrome, and other platelet secretory defects. [3],[5] Hematological evaluations in GT shows prolonged bleeding time, defective or absent clot retraction, and failure of platelets to aggregate in response to adenosine diphosphate (ADP), epinephrine, collagen, 5-hydroxytryptamine, thrombin and arachidonic acid, while coagulation tests (PTT and PT) and ristocetin-induced aggregation are normal. [3],[4] Diagnostic studies using freeze etching technique show consistent membrane abnormalities. Tests using monoclonal antibodies too are highly sensitive and specific in demonstrating the thrombasthenic defect in the platelet membrane. [3]

Most of the spontaneous bleeding episodes in GT can be treated with local measures and/or antifibrinolytic drugs. Rarely does the need of systemic hemostatic measures like platelet transfusion arise. [6] But in surgical cases, preoperative transfusion of platelets should be done to avoid excessive hemorrhage during surgery. It is also important to anticipate the risk of postsurgical hemorrhage, since bleeding in GT is unpredictable. Literature says transfusions should be continued to maintain hemostasis until wound healing is complete. [3],[7] Appliances for clot stabilization are not indicated in the treatment of bleeding disorders, since it can easily initiate more bleeding or camouflage existing bleeding. [8] Compression, gelatine sponge, etc., can be used as adjuncts to control bleed from the surgical site. [1],[2],[5]

Therapy in GT is based on both prevention and management of specific bleeding episodes. Maintenance of good oral hygiene reduces the chance of gingival hemorrhage. [1],[4],[5] Medications that affect platelet function like aspirin, NSAIDs, anticoagulants like heparin and warfarin, clopidogrel/triclopidine, abciximab (GP IIb and IIIa antagonist), streptokinase/urokinase, volume expander like dextran and dipyridamole should be avoided. Since these patients require multiple transfusions in a lifetime, measures to avoid platelet alloimmunization should be taken, which is best accomplished by leukocyte-depleted blood products and use of human leukocyte antigen (HLA)-matched platelets. Infection risks associated with multiple transfusions warrants the need of Hepatitis B vaccination. [5],[7] Antifibrinolytic drugs such as tranexamic acid or ε-aminocaproic acid can be used as an adjunct to platelet transfusion.[1],[2],[5] Desmopressin (1-deamino-8-D-arginine vasopressin (DDAVP)) do not show much clinical efficacy, though may shorten bleeding time in some patients. [1],[3] Research shows that recombinant factor VII a (rFVIIa) given as bolus injections in conjunction with antifibrinolytic agents can be a safe and mostly effective alternative to platelet transfusion for prevention and management of hemorrhage in GT, especially in those patients with antiplatelet antibodies and/or refractoriness to platelet transfusion. [6] The only curative treatment for GT mentioned in literature is hematopoietic stem cell transplantation (HSCT). But because of the risks associated with HSCT, it is recommended only in severe hemorrhages or when the patient develops refractoriness to platelet transfusion due to antibody formation. [9]


   Conclusion Top


Patients with GT shows signs of bleeding very early in life. Bleeding can be initiated by a seemingly harmless event like vaccination or tooth eruption. Once diagnosis is done, caregivers should be informed about precautions like keeping the nails short; protection from sports trauma using mouth guards, helmets, knee caps, etc.; protection from insect bites; and regular dental reviews to prevent dental problems. In girls, medical management is warranted for unavoidable hemorrhagic episodes as she goes through different stages of life like puberty and child bearing. In the event of surgical care, a hematologist should confirm the diagnosis and make the transfusion recommendations.

 
   References Top

1.Bhatia R, Mehta ND. Dental considerations in the Management of Glanzmann's Thrombasthenia. Int J Clin Pediatr Dent 2010;3:51-6.  Back to cited text no. 1
    
2.Lupien G, Amesse C, Bisonette D, Lacroix S. Glanzmann Thrombasthenia - An inherited Bleeding Disorder. In: Page D, editor. An Information Booklet, Vol. 1, 1 st ed. Canada: Canadian Association of Nurses in Heamophilia Care; 2001. p. 4-14.  Back to cited text no. 2
    
3.Levine PS. Qualitative disorders of platelet function. In: Pine WJ Jr, editor. Wintrobe's Clinical Heamatology. Vol. 2, 10 th ed. Maryland: Lippincott Williams & Wilkins; 1998. p. 1663-6.  Back to cited text no. 3
    
4.Yadalam U, Kranti K, Seshan H. Periodontal considerations for Glanzmann thrombasthenic patient. J Indian Soc Periodontol 2008;12:26-7.  Back to cited text no. 4
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5.Poornima G, Shivprasad S, Ashok L. Glanzmann's Thrombasthenia associated with a HBs Ag positive child: A case report. J Indian Soc Pedod Prev Dent 2007;25 Suppl:S37-40.  Back to cited text no. 5
    
6.Poon MC, D'Oiron R, Von Depka M, Khair K, Négrier C, Karafoulidou A, et al. International Data Collection on Recombinant Factor VIIa and Congenital Platelet Disorders Study Group. Prophylactic and therapeutic recombinant factor VIIa administration to patients with Glanzmann's Thrombasthenia: Results of an international survey. J Thromb Haemost 2004;2:1096-103.  Back to cited text no. 6
    
7.Glanzmann Thrombasthenia [Internet] Glanzmann's Research Foundation; 1990. Available from: http://www.glanzmanns.com/medArticles.htm [Last updated 2000 Dec 14; Last cited 2013 Nov 11].  Back to cited text no. 7
    
8.Berlocher CW, Zeltzer MP. Glanzmann's Thrombasthenia: Prevention of bleeding complications using microfibrillar collagen and oral stints. Pediatr Dent 1982;4:343-6.  Back to cited text no. 8
    
9.Ishaqi MK, El-Hayek M, Gassas A, Khanani M, Trad O, Baroudi M, et al. Allogenic stem cell transplantation for Glanzmann's Thrombasthenia. Pediatr Blood Cancer 2009;52:682-3.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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