Glanzmann's thrombasthenia differential diagnosis: Difference between revisions

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{{Glanzmann's thrombasthenia}}
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Glanzmann%27s_thrombasthenia]]


{{CMG}} '''Associate Editor(s)-in-Chief:''' [[User:Niush.D|Niyousha Danesh, MD-MPH]]
{{CMG}}, {{AE}} {{OK}}, [[User:Niush.D|Niyousha Danesh, MD-MPH]]


==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]].
Glanzman’s  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 Diagnoses ==
* platelet Disorders (like : [[Bernard-Soulier syndrome]],platelet storage pool defects,platelet-type [[von Willebrand disease]] and [[gray platelet syndrome]]),  
* Platelet Disorders (like : [[Bernard-Soulier syndrome]],platelet storage pool defects,platelet-type [[von Willebrand disease]] and [[gray platelet syndrome]])<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>[[Bernard-Soulier Syndrome]]<ref name="pmid18081445">{{cite journal| author=Pham A, Wang J| title=Bernard-Soulier syndrome: an inherited platelet disorder. | journal=Arch Pathol Lab Med | year= 2007 | volume= 131 | issue= 12 | pages= 1834-6 | pmid=18081445 | doi=10.1043/1543-2165(2007)131[1834:BSAIPD]2.0.CO;2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18081445  }}</ref>
* [[Von Willebrand Disease]] and
 
* [[Wiskott-Aldrich Syndrome]].
* [[Von Willebrand Disease]]
* [[Fibrinogen]] abnormalities ,(eg [[Afibrinogenemia]])
* [[Wiskott-Aldrich Syndrome]]
* [[Fibrinogen]] abnormalities(eg [[Afibrinogenemia]])


==Differential Diagnosis==
==Differential Diagnosis==
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[[Category:Disease]]
[[Category:Disease]]
[[Category:Hematology]]
[[Category:Hematology]]
[[Category:Primary care]]

Latest revision as of 21:52, 29 July 2020

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Omer Kamal, M.D.[2], Niyousha Danesh, MD-MPH

Overview

Glanzman’s  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 fatal bleeding Epistaxis Oral bleeding Menorrhagia & Postpartum hemorrhage Infection Hemarthrosis Additional

information

Glanzmann Thrombasthenia[3] ↑ Normal (Nl) Nl lower level of Normal Nl + + + + + + + _ _ Autosomal recessive (AR)

GpIIb/IIIa receptor defect

ITGA2B and ITGB3 gene

  • Platelet aggregation responds to ristocetin but not (ADP), epinephrine, and collagen.
  • Local hemostatic procedures
  • Anti-fibrinolytic drugs
  • rFVIIa, IVIG, plasmapheresis, corticosteroids, rituximab
  • HSCT[4]
Von Willebrand disease (vWD) Nl Nl ↑ Nl ↓ + + + + + + + _ _ Autosomal dominant (AD) and autosomal recessive, AR (rare)
  • vWF
  • DDAVP
  • factor VIII concentrates[1]
Bernard-Soulier Syndrome[2] ↑ ↓ Nl ↓

giant platelets

↑ + + + + + + + _ _ AR

↓GPIb/IX/V platelets

  • platelets do not aggregate in response to ristocetin but responds to ADP, epinephrine, and collagen
  • DDAVP
  • antifibrinolytic
  • rFVIIa[5]
Wiskott-Aldrich Syndrome[6] Nl Nl Nl ↓

microthrombocytopenia

Nl + + ↓ +

(specially GI bleeding)

+ ↓ ↓ + _
  • X-linked defect in The WASp gene
  • +Severe eczema
  • antibiotics, antivirals, antifungals,
  • chemotherapy
  • immunoglobulins
  • corticosteroids
Inherited Abnormalities of Fibrinogen[7] ↑ ↑ ↑ Nl ↓ Inherited Abnormalities of Fibrinogen + + + + + + _ + Fibrinogen defect

3gene:FGA, FGB, and FGG

  • Fibrinogen concentrates
  • cryoprecipitate[8]

References

  1. ↑ 1.0 1.1 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.
  2. ↑ 2.0 2.1 Pham A, Wang J (2007). "Bernard-Soulier syndrome: an inherited platelet disorder". Arch Pathol Lab Med. 131 (12): 1834–6. doi:10.1043/1543-2165(2007)131[1834:BSAIPD]2.0.CO;2. PMID 18081445.
  3. ↑ Nurden AT (2006). "Glanzmann thrombasthenia". Orphanet J Rare Dis. 1: 10. doi:10.1186/1750-1172-1-10. PMC 1475837. PMID 16722529.
  4. ↑ 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.
  5. ↑ Diz-Küçükkaya R (2013). "Inherited platelet disorders including Glanzmann thrombasthenia and Bernard-Soulier syndrome". Hematology Am Soc Hematol Educ Program. 2013: 268–75. doi:10.1182/asheducation-2013.1.268. PMID 24319190.
  6. ↑ Ochs HD, Thrasher AJ (2006). "The Wiskott-Aldrich syndrome". J Allergy Clin Immunol. 117 (4): 725–38, quiz 739. doi:10.1016/j.jaci.2006.02.005. PMID 16630926.
  7. ↑ Zhou J, Ding Q, Chen Y, Ouyang Q, Jiang L, Dai J; et al. (2015). "Clinical features and molecular basis of 102 Chinese patients with congenital dysfibrinogenemia". Blood Cells Mol Dis. 55 (4): 308–15. doi:10.1016/j.bcmd.2015.06.002. PMID 26460252.
  8. ↑ Verhovsek M, Moffat KA, Hayward CP (2008). "Laboratory testing for fibrinogen abnormalities". Am J Hematol. 83 (12): 928–31. doi:10.1002/ajh.21293. PMID 18951466.
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