Glanzmann's thrombasthenia differential diagnosis: Difference between revisions
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==Overview== | ==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 Diagnoses == | ||
* | * Platelet Disorders (like : [[Bernard-Soulier syndrome]],platelet storage pool defects,platelet-type [[von Willebrand disease]] and [[gray platelet syndrome]]), | ||
* [[Von Willebrand Disease]] | * [[Von Willebrand Disease]] | ||
* [[Wiskott-Aldrich Syndrome]] | * [[Wiskott-Aldrich Syndrome]] | ||
* [[Fibrinogen]] abnormalities | * [[Fibrinogen]] abnormalities(eg [[Afibrinogenemia]]) | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Revision as of 18:53, 13 November 2018
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Glanzmann's thrombasthenia |
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Differentiating Glanzmann's thrombasthenia from other Diseases |
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Diagnosis |
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Treatment |
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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
- Platelet Disorders (like : Bernard-Soulier syndrome,platelet storage pool defects,platelet-type von Willebrand disease and gray platelet syndrome),
- Von Willebrand Disease
- Wiskott-Aldrich Syndrome
- Fibrinogen abnormalities(eg Afibrinogenemia)
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[1] | ↑ | Normal (Nl) | Nl | lower level of Normal | Nl | + | + | + | + | + | + | + | _ | _ | Autosomal recessive (AR)
GpIIb/IIIa receptor defect ITGA2B and ITGB3 gene
|
|
| Von Willebrand disease (vWD) | Nl | Nl | ↑ | Nl | ↓ | + | + | + | + | + | + | + | _ | _ | Autosomal dominant (AD) and autosomal recessive, AR (rare) |
|
| Bernard-Soulier Syndrome[4] | ↑ | ↓ | Nl | ↓
giant platelets |
↑ | + | + | + | + | + | + | + | _ | _ | AR
↓GPIb/IX/V platelets
|
|
| Wiskott-Aldrich Syndrome[6] | Nl | Nl | Nl | ↓
microthrombocytopenia |
Nl | + | + | ↓ | +
(specially GI bleeding) |
+ | ↓ | ↓ | + | _ |
|
|
| Inherited Abnormalities of Fibrinogen[7] | ↑ | ↑ | ↑ | Nl | ↓ | Inherited Abnormalities of Fibrinogen | + | + | + | + | + | + | _ | + | Fibrinogen defect
3gene:FGA, FGB, and FGG |
|
References
- ↑ Nurden AT (2006). "Glanzmann thrombasthenia". Orphanet J Rare Dis. 1: 10. doi:10.1186/1750-1172-1-10. PMC 1475837. PMID 16722529.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.