Glanzmann's thrombasthenia differential diagnosis: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
No edit summary |
||
| Line 8: | Line 8: | ||
== 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]] | * [[Von Willebrand Disease]] | ||
* [[Wiskott-Aldrich Syndrome]] | * [[Wiskott-Aldrich Syndrome]] | ||
Revision as of 19:09, 13 November 2018
|
Glanzmann's thrombasthenia |
|
Differentiating Glanzmann's thrombasthenia from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Glanzmann's thrombasthenia differential diagnosis On the Web |
|
American Roentgen Ray Society Images of Glanzmann's thrombasthenia differential diagnosis |
|
Glanzmann's thrombasthenia differential diagnosis in the news |
|
Risk calculators and risk factors for Glanzmann's thrombasthenia differential diagnosis |
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)[1]
|- |Bernard-Soulier Syndrome[2]
- 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[3] | ↑ | 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[2] | ↑ | ↓ | 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
- ↑ 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.
- ↑ 2.0 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.
- ↑ 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.
- ↑ 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.