Glanzmann's thrombasthenia differential diagnosis: Difference between revisions

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==Overview==
==Overview==
Glanzmann thrombasthenia must be differentiated from other diseases that cause severe hemorrhages , mucocutaneous bleeding , petechiae and ecchymosis, such as Platelet Disorders (like : Bernard-Soulier syndrome,platelet storage pool defects,platelet-type von Willebrand disease and gray platelet syndrome), Fibrinogen abnormalities ,(eg Afibrinogenemia), Von Willebrand Disease and wiskott-Aldrich Syndrome.
== Differential Diagnoses ==
*
*
*


==Differential Diagnosis==
==Differential Diagnosis==
{| class="wikitable"
|-
! rowspan="2" |Diseases
! colspan="5" |Laboratory Findings
! colspan="4" |Physical Examination
! colspan="4" |History and Symptoms
! rowspan="2" |Treatment
|-
!Bleeding Time (BT)
!PT
!aPTT
!Platelet count
!vWF
!Petechiae
& Purpura
!Ecchymosis
!Mucocutaneous hemorrhage
!Severe bleeding
!Epistaxis
!Oral bleeding
![[Menorrhagia]] & [[Postpartum hemorrhage]]
!Hemarthrosis
|-
|Glanzmann Thrombasthenia<ref name="pmid16722529" />
|'''↑'''
|Normal (Nl)
|Nl
|lower level of Normal
|Nl
|<nowiki>+</nowiki>
| +
| +
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|_
|
* Local hemostatic procedures
* Anti-fibrinolytic drugs
* rFVIIa, IVIG, plasmapheresis, corticosteroids,  rituximab
* HSCT<ref name="pmid26185478">{{cite journal| author=Solh T, Botsford A, Solh M| title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. | journal=J Blood Med | year= 2015 | volume= 6 | issue=  | pages= 219-27 | pmid=26185478 | doi=10.2147/JBM.S71319 | pmc=4501245 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26185478  }}</ref>
|-
|[[Von Willebrand disease]] ('''vWD)'''
|Nl
|Nl
|'''↑'''
|Nl
|↓
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| +
| +
| +
| +
| +
|_
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* vWF
* Desmopressin
* factor VIII concentrates<ref name="pmid22918553" />
|-
|Platelet disorder
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|↓
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{| class="wikitable"
|+
!Variable
!Glanzmann Thrombasthenia
![[Von Willebrand disease]] ('''vWD)'''
!
!
!Platelet disorder
|-
|Pathophysiology
|Autosomal recessive (AR)
[[GpIIb/IIIa]] receptor defect
ITGA2B and ITGB3 gene
|Autosomal dominant (AD) and autosomal recessive, AR (rare)
|
|
|Variable depending of type
|-
|History &
Physical examination
|Ecchymosis
* Epistaxis
* gingival Bleeding 
* Prolonged bleeding with minor injuries
* Heavy menstrual bleeding
* postpartum hemorrhage
* gastrointestinal bleeding
* Heavy bleeding during and after surgery <ref name="pmid16722529">{{cite journal| author=Nurden AT| title=Glanzmann thrombasthenia. | journal=Orphanet J Rare Dis | year= 2006 | volume= 1 | issue=  | pages= 10 | pmid=16722529 | doi=10.1186/1750-1172-1-10 | pmc=1475837 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16722529  }}</ref>
|
* Epistaxis
* Ecchymosis  .
* [[Menorrhagia|Heavy menstrual periods ,]]
* [[Postpartum hemorrhage]]
* [[Mucocutaneaus hemorrhage|Mucocutaneous hemorrhage]]
* Bleeding after surgery in adults.
|
|
|Superficial bleeding
|-
|Labratory findings
|
{| class="wikitable"
|Bleeding Time (BT)
|Prolonged
|-
|Prothrobin time
|Normal
|-
|aPTT
|Normal
|-
|Platelet count
|lower level of Normal
|-
|vWF
|Normal
|}
|
|
|
|
|-
|Bleeding Time (BT)
|Prolonged
|
|
|
|
|-
|Prothrobin time
|Normal
|Normal
|
|
|Normal
|-
|aPTT
|Normal
|Prolonged
|
|
|Normal
|-
|Platelet count
|lower level of Normal
|Normal
|
|
|Low
|-
|vWF
|Normal
|Decreased
|
|
|Normal
|-
|Treatment
|Local hemostatic procedures
Anti-fibrinolytic drugs
rFVIIa, IVIG, plasmapheresis, systemic corticosteroids, protein A Sepharose immunoadsorption, cyclophosphamide, azathioprine, chemotherapy and rituximab
HSCT
|vWF
Desmopressin
factor VIII concentrates<ref name="pmid22918553">{{cite journal| author=de Wee EM, Sanders YV, Mauser-Bunschoten EP, van der Bom JG, Degenaar-Dujardin ME, Eikenboom J et al.| title=Determinants of bleeding phenotype in adult patients with moderate or severe von Willebrand disease. | journal=Thromb Haemost | year= 2012 | volume= 108 | issue= 4 | pages= 683-92 | pmid=22918553 | doi=10.1160/TH12-04-0244 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22918553  }}</ref>
|
|
|Depends on type
|}


==References==
==References==

Revision as of 10:49, 20 July 2018

Glanzmann's thrombasthenia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Glanzmann thrombasthenia must be differentiated from other diseases that cause severe hemorrhages , mucocutaneous bleeding , petechiae and ecchymosis, such as Platelet Disorders (like : Bernard-Soulier syndrome,platelet storage pool defects,platelet-type von Willebrand disease and gray platelet syndrome), Fibrinogen abnormalities ,(eg Afibrinogenemia), Von Willebrand Disease and wiskott-Aldrich Syndrome.

Differential Diagnoses

Differential Diagnosis

Diseases Laboratory Findings Physical Examination History and Symptoms Treatment
Bleeding Time (BT) PT aPTT Platelet count vWF Petechiae

& Purpura

Ecchymosis Mucocutaneous hemorrhage Severe bleeding Epistaxis Oral bleeding Menorrhagia & Postpartum hemorrhage Hemarthrosis
Glanzmann Thrombasthenia[1] ↑ Normal (Nl) Nl lower level of Normal Nl + + + + + + + _
  • Local hemostatic procedures
  • Anti-fibrinolytic drugs
  • rFVIIa, IVIG, plasmapheresis, corticosteroids, rituximab
  • HSCT[2]
Von Willebrand disease (vWD) Nl Nl ↑ Nl ↓ + + + + + + + _
  • vWF
  • Desmopressin
  • factor VIII concentrates[3]
Platelet disorder ↓
Variable Glanzmann Thrombasthenia Von Willebrand disease (vWD) Platelet disorder
Pathophysiology Autosomal recessive (AR)

GpIIb/IIIa receptor defect

ITGA2B and ITGB3 gene

Autosomal dominant (AD) and autosomal recessive, AR (rare) Variable depending of type
History &

Physical examination

Ecchymosis
  • Epistaxis
  • gingival Bleeding
  • Prolonged bleeding with minor injuries
  • Heavy menstrual bleeding
  • postpartum hemorrhage
  • gastrointestinal bleeding
  • Heavy bleeding during and after surgery [1]
  • Epistaxis
  • Ecchymosis .
 Superficial bleeding
Labratory findings
Bleeding Time (BT) Prolonged
Prothrobin time Normal
aPTT Normal
Platelet count lower level of Normal
vWF Normal
Bleeding Time (BT) Prolonged
Prothrobin time Normal Normal Normal
aPTT Normal Prolonged Normal
Platelet count lower level of Normal Normal Low
vWF Normal Decreased Normal
Treatment Local hemostatic procedures

Anti-fibrinolytic drugs

rFVIIa, IVIG, plasmapheresis, systemic corticosteroids, protein A Sepharose immunoadsorption, cyclophosphamide, azathioprine, chemotherapy and rituximab

HSCT

vWF

Desmopressin

factor VIII concentrates[3]

Depends on type

References

  1. ↑ 1.0 1.1 Nurden AT (2006). "Glanzmann thrombasthenia". Orphanet J Rare Dis. 1: 10. doi:10.1186/1750-1172-1-10. PMC 1475837. PMID 16722529.
  2. ↑ Solh T, Botsford A, Solh M (2015). "Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options". J Blood Med. 6: 219–27. doi:10.2147/JBM.S71319. PMC 4501245. PMID 26185478.
  3. ↑ 3.0 3.1 de Wee EM, Sanders YV, Mauser-Bunschoten EP, van der Bom JG, Degenaar-Dujardin ME, Eikenboom J; et al. (2012). "Determinants of bleeding phenotype in adult patients with moderate or severe von Willebrand disease". Thromb Haemost. 108 (4): 683–92. doi:10.1160/TH12-04-0244. PMID 22918553.
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