Glanzmann's thrombasthenia physical examination: Difference between revisions
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==Overview== | ==Overview== | ||
Patients with [[Glanzmann's thrombasthenia]] may be [[asymptomatic]], or they could manifest [[mucosal bleeding]], [[ecchymoses]], [[petechiae]] and [[purpura]] or current [[bleeding]] on [[physical exam]]. | Patients with [[Glanzmann's thrombasthenia]] may be [[asymptomatic]], or they could manifest [[mucosal bleeding]], [[ecchymoses]], [[petechiae]] and [[purpura]] or current [[bleeding]] on [[physical exam]].<ref name="pmid21917754">{{cite journal |vauthors=Nurden AT, Fiore M, Nurden P, Pillois X |title=Glanzmann thrombasthenia: a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models |journal=Blood |volume=118 |issue=23 |pages=5996–6005 |date=December 2011 |pmid=21917754 |doi=10.1182/blood-2011-07-365635 |url=}}</ref><ref name="pmid30117143">{{cite journal |vauthors=Grainger JD, Thachil J, Will AM |title=How we treat the platelet glycoprotein defects; Glanzmann thrombasthenia and Bernard Soulier syndrome in children and adults |journal=Br. J. Haematol. |volume=182 |issue=5 |pages=621–632 |date=September 2018 |pmid=30117143 |doi=10.1111/bjh.15409 |url=}}</ref><ref name="pmid30097224">{{cite journal |vauthors=Bannow BS, Konkle BA |title=Inherited Bleeding Disorders in the Obstetric Patient |journal=Transfus Med Rev |volume=32 |issue=4 |pages=237–243 |date=October 2018 |pmid=30097224 |doi=10.1016/j.tmrv.2018.06.003 |url=}}</ref><ref name="pmid30078718">{{cite journal |vauthors=Barg AA, Hauschner H, Misgav M, Lubetsky A, Levy-Mendelowitz S, Livnat T, Avishai E, Rosenberg N, Kenet G |title=A novel approach using ancillary tests to guide treatment of Glanzmann thrombasthenia patients undergoing surgical procedures |journal=Blood Cells Mol. Dis. |volume=72 |issue= |pages=44–48 |date=September 2018 |pmid=30078718 |doi=10.1016/j.bcmd.2018.07.003 |url=}}</ref><ref name="pmid30032577">{{cite journal |vauthors=Zhou L, Wang ZY, Ruan CG |title=[Special type of Glanzmann's thrombasthenia] |language=Chinese |journal=Zhonghua Xue Ye Xue Za Zhi |volume=39 |issue=6 |pages=524–526 |date=June 2018 |pmid=30032577 |doi=10.3760/cma.j.issn.0253-2727.2018.06.021 |url=}}</ref><ref name="pmid30031712">{{cite journal |vauthors=Lee A, Poon MC |title=Inherited platelet functional disorders: General principles and practical aspects of management |journal=Transfus. Apher. Sci. |volume=57 |issue=4 |pages=494–501 |date=August 2018 |pmid=30031712 |doi=10.1016/j.transci.2018.07.010 |url=}}</ref><ref name="pmid29985398">{{cite journal |vauthors=Blair TA, Michelson AD, Frelinger AL |title=Mass Cytometry Reveals Distinct Platelet Subtypes in Healthy Subjects and Novel Alterations in Surface Glycoproteins in Glanzmann Thrombasthenia |journal=Sci Rep |volume=8 |issue=1 |pages=10300 |date=July 2018 |pmid=29985398 |pmc=6037710 |doi=10.1038/s41598-018-28211-5 |url=}}</ref><ref name="pmid29945125">{{cite journal |vauthors=Buckley F, Norris A, Kerr R |title=Management of Abdominoperineal Excision of the Rectum in a Patient with Glanzmann Thrombasthenia |journal=Acta Haematol. |volume=139 |issue=4 |pages=243–246 |date=2018 |pmid=29945125 |doi=10.1159/000489114 |url=}}</ref> | ||
==Physical examination== | ==Physical examination== |
Revision as of 17:47, 10 December 2018
Glanzmann's thrombasthenia |
Differentiating Glanzmann's thrombasthenia from other Diseases |
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Diagnosis |
Treatment |
Case Studies |
Glanzmann's thrombasthenia physical examination On the Web |
American Roentgen Ray Society Images of Glanzmann's thrombasthenia physical examination |
Risk calculators and risk factors for Glanzmann's thrombasthenia physical examination |
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
Patients with Glanzmann's thrombasthenia may be asymptomatic, or they could manifest mucosal bleeding, ecchymoses, petechiae and purpura or current bleeding on physical exam.[1][2][3][4][5][6][7][8]
Physical examination
Appearance of the Patient
- Patient appears well in the absence of severe hemorrhage
- Patient appears ill in the persistent bleeding.
Vital Signs
- May haveTachycardia, depends on the severity of the bleeding
- May haveTachypnea depends on severity of the bleeding
Skin
- Cyanosis
- Pallor
- Secondary to anemia
- Ecchymoses,petechiae and purpura
- Secondary to thrombocytopenia
HEENT
- Evidence of trauma
- Pale conjunctiva
- Epistaxis
- Palatal petechiae
- Gingival or other oral cavity bleeding
Neck
No specific signs
Lungs
- Commonly normal lung sounds
Heart
- Commonly normal heart sounds
Abdomen
The spleen is usually non palpable
Neuromuscular
- Patient is usually oriented to persons, place, and time
- Glasgow coma scale is normally 15 / 15 but may may present loss of consciousness in severe fatal hemorrhage episodes [9]
Gallery
Extremities
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Glanzmann’s trombastenia. With permission from Dermatology Atlas.[10]
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Glanzmann’s trombastenia. With permission from Dermatology Atlas.[10]
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Glanzmann’s trombastenia. With permission from Dermatology Atlas.[10]
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Glanzmann’s trombastenia. With permission from Dermatology Atlas.[10]
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Glanzmann’s trombastenia. With permission from Dermatology Atlas.[10]
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Glanzmann’s trombastenia. With permission from Dermatology Atlas.[10]
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Glanzmann’s trombastenia. With permission from Dermatology Atlas.[10]
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Glanzmann’s trombastenia. With permission from Dermatology Atlas.[10]
References
- ↑ Nurden AT, Fiore M, Nurden P, Pillois X (December 2011). "Glanzmann thrombasthenia: a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models". Blood. 118 (23): 5996–6005. doi:10.1182/blood-2011-07-365635. PMID 21917754.
- ↑ Grainger JD, Thachil J, Will AM (September 2018). "How we treat the platelet glycoprotein defects; Glanzmann thrombasthenia and Bernard Soulier syndrome in children and adults". Br. J. Haematol. 182 (5): 621–632. doi:10.1111/bjh.15409. PMID 30117143.
- ↑ Bannow BS, Konkle BA (October 2018). "Inherited Bleeding Disorders in the Obstetric Patient". Transfus Med Rev. 32 (4): 237–243. doi:10.1016/j.tmrv.2018.06.003. PMID 30097224.
- ↑ Barg AA, Hauschner H, Misgav M, Lubetsky A, Levy-Mendelowitz S, Livnat T, Avishai E, Rosenberg N, Kenet G (September 2018). "A novel approach using ancillary tests to guide treatment of Glanzmann thrombasthenia patients undergoing surgical procedures". Blood Cells Mol. Dis. 72: 44–48. doi:10.1016/j.bcmd.2018.07.003. PMID 30078718.
- ↑ Zhou L, Wang ZY, Ruan CG (June 2018). "[Special type of Glanzmann's thrombasthenia]". Zhonghua Xue Ye Xue Za Zhi (in Chinese). 39 (6): 524–526. doi:10.3760/cma.j.issn.0253-2727.2018.06.021. PMID 30032577.
- ↑ Lee A, Poon MC (August 2018). "Inherited platelet functional disorders: General principles and practical aspects of management". Transfus. Apher. Sci. 57 (4): 494–501. doi:10.1016/j.transci.2018.07.010. PMID 30031712.
- ↑ Blair TA, Michelson AD, Frelinger AL (July 2018). "Mass Cytometry Reveals Distinct Platelet Subtypes in Healthy Subjects and Novel Alterations in Surface Glycoproteins in Glanzmann Thrombasthenia". Sci Rep. 8 (1): 10300. doi:10.1038/s41598-018-28211-5. PMC 6037710. PMID 29985398.
- ↑ Buckley F, Norris A, Kerr R (2018). "Management of Abdominoperineal Excision of the Rectum in a Patient with Glanzmann Thrombasthenia". Acta Haematol. 139 (4): 243–246. doi:10.1159/000489114. PMID 29945125.
- ↑ Nurden AT (2006). "Glanzmann thrombasthenia". Orphanet J Rare Dis. 1: 10. doi:10.1186/1750-1172-1-10. PMC 1475837. PMID 16722529.
- ↑ 10.0 10.1 10.2 10.3 10.4 10.5 10.6 10.7 "Dermatology Atlas".