A Case Report on Glanzmann’s Thrombasthenia: A Rare Platelet Function Disorder

2018 ◽  
Vol 2 (02) ◽  
pp. 59-60
Author(s):  
Farida Yasmin ◽  
Md. Anwarul Karim ◽  
Chowdhury Yakub Jamal ◽  
Mamtaz Begum ◽  
Ferdousi Begum

Epistaxis in children is one of the important presenting symptoms for attending emergency department in paediatric patients. Recurrent epistaxis is common in children. Although epistaxis in children usually occurred due to different benign conditions, it may be one of the important presenting symptoms of some inherited bleeding disorder. Whereas most bleeding disorders can be diagnosed through different standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. In this article we describe one case report of platelet function disorders on Glanzmann’s thrombasthenia (GT). Our patient was a 10-year old girl who presented to us with history of recurrent severe epistaxis. She had a bruise on her abdomen and many scattered petechiae in different parts of the body. Her previous investigations revealed no demonstrable haemostatic anomalies. After performing platelet aggregation test, she was diagnosed as GT.

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Michael Recht ◽  
Meera Chitlur ◽  
Derek Lam ◽  
Syana Sarnaik ◽  
Madhvi Rajpurkar ◽  
...  

Children often present to emergency departments (EDs) with uncontrollable nose bleeding. Although usually due to benign etiologies, epistaxis may be the presenting symptom of an inherited bleeding disorder. Whereas most bleeding disorders are detected through standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. Here we present two case reports and review diagnostic and management challenges of platelet function disorders with a focus on Glanzmann’s thrombasthenia (GT). Patient 1 was a 4-year-old boy with uncontrolled epistaxis. His medical history included frequent and easy bruising. Previous laboratory evaluation revealed only mild microcytic anemia. An otolaryngologist stopped the bleeding, and referral to a pediatric hematologist led to the definitive diagnosis of GT. Patient 2 was a 2.5-year-old girl with severe epistaxis and a history of milder recurrent epistaxis. She had a bruise on her abdomen with a palpable hematoma and many scattered petechiae. Previous assessments revealed no demonstrable hemostatic anomalies. Platelet aggregation studies were performed following referral to a pediatric hematologist, leading to the diagnosis of GT. As evidenced by these cases, the ED physician may often be the first to evaluate severe or recurrent epistaxis and should recognize indications for coagulation testing and hematology consultation/referral for advanced hematologic assessments.


F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 1219
Author(s):  
Prashant Pant ◽  
Oshan Shrestha ◽  
Pawan Budhathoki ◽  
Nebula Devkota ◽  
Prabin Kumar Giri ◽  
...  

Ludwig’s angina (LA) is a rapidly spreading and potentially life-threatening infection having an odontogenic infection as the most common source. It involves the floor of the mouth and neck. Modernization in medical care has made this entity rare and of low mortality at the present but it is still feared as a lethal entity due to rapidly progressive airway obstruction that follows. Here we report a case of a 15-year-old male who suffered from LA. Presenting symptoms and findings of the examination helped in the clinical diagnosis. Immediate intubation, use of broad-spectrum antibiotics, and treatment of complications aided the patient’s recovery. LA should be considered for differential diagnosis in the case of neck swelling especially in those having a history of poor oral hygiene and recent dental procedures. Management of LA and its complications should always involve doctors from multiple disciplines.


2007 ◽  
Vol 53 (3) ◽  
pp. 407
Author(s):  
Min-Woo Jeong ◽  
Kwang-Suk Seo ◽  
Hyun-Jeong Kim ◽  
Kwang-Won Yum

1970 ◽  
Vol 15 (2) ◽  
pp. 75-77
Author(s):  
M Alamgir Chowdhury ◽  
Mousumi Malakar ◽  
SM Golam Rabbani ◽  
Naseem Yasmeen ◽  
Shahidul Islam

Juvenile nasopharyngeal angiofibroma is a benign vascular neoplasm, but it is locally aggressive. This accounts for less than 0.5% of all the neoplasm in the head & neck region in the male population only. Here we report a case of 10-year-old boy with a blackish red smooth polypoidal mass in the nasal cavity, with history of recurrent epistaxis. On physical examination it was suspected as nasopharyngeal angiofibroma. We removed it totally by lateral rhinotomy approach. And the diagnosis was nasopharyngeal angiofibroma on histopathology. Key words: Angiofibroma; Nasopharyngeal. DOI: 10.3329/bjo.v15i2.5061 Bangladesh J Otorhinolaryngol 2009; 15(2): 75-77


1979 ◽  
Author(s):  
E.F. von Leeuwen ◽  
G.T.E. Zonneveld ◽  
L.E. von Riesz ◽  
C.S.P Jenkins ◽  
J.A. van Mourik ◽  
...  

The expression of the platelet-speciftc alloantigens on the platelets from 6 patients with Glanzmann’s Thrombasthenia (G.T.) and their nearest relatives was studied. The alloantigens Zwa (PIAl) and Zwb(PIA2) were found to be completely absent from thrombasthenic platelets while the alloantigens of the Ko-system were found to be normally expressed. The alloantigen Baka(phenotypefrequency 90.2%) was absent on the platelets from 4 studied G.T. patients. The platelets of all the family members reacted positively with anti-Zwa, negatively with antt-Zwb serum. SDS-PA gel electrophoresis of G.T. platelet membranes demonstrated a marked deficiency of the glycoproteins IIb and IIIa. Glycoprotein analysis of the platelet membranes from the family members of 3 of the 6 patients reveoled no apparent abnormalities.Pre-incubation with anti-Zwa containing plasma strongly inhibits ADP-and collagen induced aggregation of platelets from normal Zwa homozygous individuols with a slight inhibition of the aggregation induced by ristocetin. Zwa antibodies did not affect the functions of platelets from ZWb homozygous individuals. Thus binding of Zwa antibodies induces a thrombosthenis-like state.


2016 ◽  
Vol 44 (3) ◽  
pp. 249-254
Author(s):  
Sergio Hernando Cabarique-Serrano ◽  
Víctor Hugo González-Cárdenas ◽  
Jean Pierre Dussán-Crosby ◽  
Rodolfo Enrique Páez-González ◽  
María Alejandra Ramírez

2013 ◽  
Vol 131 ◽  
pp. S90
Author(s):  
K. Meghan ◽  
K. Ryan ◽  
C. Regan ◽  
B. Byrne ◽  
C. Lynch

2004 ◽  
Vol 19 (3) ◽  
pp. 185-185 ◽  
Author(s):  
Vittorio Di Michele ◽  
Francesca Bolino

The treatment of depressive symptoms in patients affected by schizophrenia is often a concern for clinicians [2] due to potential interaction, in terms of safety and efficacy. Citalopram seems to be a safe SSRI as adjunctive treatment to Olanzapine because of the lack of interactions. We report a serendipitous finding showing that the adjunction of Citalopram to Olanzapine, led to disappearence of residual hallucinations and depersonalization symptoms in a few weeks.Mr A is a 27-year-old male, living in the community, with a 4-year history of schizophrenia,. He was treated with low doses of Olanzapine (10 mg/daily) since 2000 because of an intolerance to dose increments (weight gain and mydriasis).The persistence of sporadic hallucinations (commenting voices) and depersonalization symptoms (when he walked alone he felt his self leaving the body) was well tolerated by the patient. The social functioning was satisfactory: he had a protected job and was involved in a comprehensive rehabilitation programme.


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