Bleeding diathesis: Difference between revisions

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! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding
! align="center" style="background:#4479BA; color: #FFFFFF;" + |<nowiki>Petechia|Petechiae</nowiki>
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia
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| align="left" style="background:#F5F5F5;" |  
| align="left" style="background:#F5F5F5;" |  
* History of prior infection
* History of prior infection
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" |+
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" |+
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" |+
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" |+
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" |+
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" |+
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | <nowiki>-</nowiki>
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! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Medication|Medications]]-Induced [[Thrombocytopenia|thrombocy]]<span name="harr_c115s002s001s003p001"></span><span name="9100757"></span>[[Thrombocytopenia|topenia]] <ref name="pmid25134884">{{cite journal |vauthors=Kam T, Alexander M |title=Drug-induced immune thrombocytopenia |journal=J Pharm Pract |volume=27 |issue=5 |pages=430–9 |date=October 2014 |pmid=25134884 |doi=10.1177/0897190014546099 |url=}}</ref><ref name="pmid23461497">{{cite journal |vauthors=Seco-Melantuche R, Delgado-Sánchez O, Álvarez-Arroyo L |title=[Incidence of drug-induced thrombocytopenia in hospitalized patients] |language=Spanish; Castilian |journal=Farm Hosp |volume=37 |issue=1 |pages=27–34 |date=2013 |pmid=23461497 |doi=10.7399/FH.2013.37.1.42 |url=}}</ref>
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Medication|Medications]]-Induced [[Thrombocytopenia|thrombocy]]<span name="harr_c115s002s001s003p001"></span><span name="9100757"></span>[[Thrombocytopenia|topenia]] <ref name="pmid25134884">{{cite journal |vauthors=Kam T, Alexander M |title=Drug-induced immune thrombocytopenia |journal=J Pharm Pract |volume=27 |issue=5 |pages=430–9 |date=October 2014 |pmid=25134884 |doi=10.1177/0897190014546099 |url=}}</ref><ref name="pmid23461497">{{cite journal |vauthors=Seco-Melantuche R, Delgado-Sánchez O, Álvarez-Arroyo L |title=[Incidence of drug-induced thrombocytopenia in hospitalized patients] |language=Spanish; Castilian |journal=Farm Hosp |volume=37 |issue=1 |pages=27–34 |date=2013 |pmid=23461497 |doi=10.7399/FH.2013.37.1.42 |url=}}</ref>
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** [[Linezolid]]  
** [[Linezolid]]  
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| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" |+
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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* Unexplained [[thrombocytopenia]] up to 3 weeks after the end of [[heparin]] therapy
* Unexplained [[thrombocytopenia]] up to 3 weeks after the end of [[heparin]] therapy
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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* History of prior [[infection]] or no history
* History of prior [[infection]] or no history
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
|-
|-
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited [[Thrombocytopenia]]<span name="harr_c115s002s001s006p001"></span><span name="9100783"></span><ref name="pmid27025194">{{cite journal |vauthors=Johnson B, Fletcher SJ, Morgan NV |title=Inherited thrombocytopenia: novel insights into megakaryocyte maturation, proplatelet formation and platelet lifespan |journal=Platelets |volume=27 |issue=6 |pages=519–25 |date=September 2016 |pmid=27025194 |pmc=5000870 |doi=10.3109/09537104.2016.1148806 |url=}}</ref><ref name="pmid30103613">{{cite journal |vauthors=Wang Q, Cao L, Sheng G, Shen H, Ling J, Xie J, Ma Z, Yin J, Wang Z, Yu Z, Chen S, Zhao Y, Ruan C, Xia L, Jiang M |title=Application of High-Throughput Sequencing in the Diagnosis of Inherited Thrombocytopenia |journal=Clin. Appl. Thromb. Hemost. |volume= |issue= |pages=1076029618790696 |date=August 2018 |pmid=30103613 |doi=10.1177/1076029618790696 |url=}}</ref>
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited [[Thrombocytopenia]]<span name="harr_c115s002s001s006p001"></span><span name="9100783"></span><ref name="pmid27025194">{{cite journal |vauthors=Johnson B, Fletcher SJ, Morgan NV |title=Inherited thrombocytopenia: novel insights into megakaryocyte maturation, proplatelet formation and platelet lifespan |journal=Platelets |volume=27 |issue=6 |pages=519–25 |date=September 2016 |pmid=27025194 |pmc=5000870 |doi=10.3109/09537104.2016.1148806 |url=}}</ref><ref name="pmid30103613">{{cite journal |vauthors=Wang Q, Cao L, Sheng G, Shen H, Ling J, Xie J, Ma Z, Yin J, Wang Z, Yu Z, Chen S, Zhao Y, Ruan C, Xia L, Jiang M |title=Application of High-Throughput Sequencing in the Diagnosis of Inherited Thrombocytopenia |journal=Clin. Appl. Thromb. Hemost. |volume= |issue= |pages=1076029618790696 |date=August 2018 |pmid=30103613 |doi=10.1177/1076029618790696 |url=}}</ref>
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* Positive family history
* Positive family history
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
|-
|-
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombotic thrombocytopenic purpura|Thrombotic Thrombocytopenic Purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]])<span name="harr_c115s002s002s001p001"></span><span name="9100787"></span><ref name="pmid30220931">{{cite journal |vauthors=Knöbl P |title=Thrombotic thrombocytopenic purpura |journal=Memo |volume=11 |issue=3 |pages=220–226 |date=2018 |pmid=30220931 |doi=10.1007/s12254-018-0429-6 |url=}}</ref><ref name="pmid26386489">{{cite journal |vauthors=Mannucci PM, Cugno M |title=The complex differential diagnosis between thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: Laboratory weapons and their impact on treatment choice and monitoring |journal=Thromb. Res. |volume=136 |issue=5 |pages=851–4 |date=November 2015 |pmid=26386489 |doi=10.1016/j.thromres.2015.09.007 |url=}}</ref>
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombotic thrombocytopenic purpura|Thrombotic Thrombocytopenic Purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]])<span name="harr_c115s002s002s001p001"></span><span name="9100787"></span><ref name="pmid30220931">{{cite journal |vauthors=Knöbl P |title=Thrombotic thrombocytopenic purpura |journal=Memo |volume=11 |issue=3 |pages=220–226 |date=2018 |pmid=30220931 |doi=10.1007/s12254-018-0429-6 |url=}}</ref><ref name="pmid26386489">{{cite journal |vauthors=Mannucci PM, Cugno M |title=The complex differential diagnosis between thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: Laboratory weapons and their impact on treatment choice and monitoring |journal=Thromb. Res. |volume=136 |issue=5 |pages=851–4 |date=November 2015 |pmid=26386489 |doi=10.1016/j.thromres.2015.09.007 |url=}}</ref>
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*[[HIV-1]] infection
*[[HIV-1]] infection
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
|-
|-
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemolytic-uremic syndrome|Hemolytic Uremic Syndrome]]<span name="harr_c115s002s002s002p001"></span><span name="9100796"></span><ref name="pmid24365375">{{cite journal |vauthors=Webster K, Schnitzler E |title=Hemolytic uremic syndrome |journal=Handb Clin Neurol |volume=120 |issue= |pages=1113–23 |date=2014 |pmid=24365375 |doi=10.1016/B978-0-7020-4087-0.00075-9 |url=}}</ref><ref name="pmid25845294">{{cite journal |vauthors=Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P |title=Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome |journal=Pathol. Biol. |volume=63 |issue=3 |pages=136–43 |date=June 2015 |pmid=25845294 |doi=10.1016/j.patbio.2015.03.001 |url=}}</ref>
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemolytic-uremic syndrome|Hemolytic Uremic Syndrome]]<span name="harr_c115s002s002s002p001"></span><span name="9100796"></span><ref name="pmid24365375">{{cite journal |vauthors=Webster K, Schnitzler E |title=Hemolytic uremic syndrome |journal=Handb Clin Neurol |volume=120 |issue= |pages=1113–23 |date=2014 |pmid=24365375 |doi=10.1016/B978-0-7020-4087-0.00075-9 |url=}}</ref><ref name="pmid25845294">{{cite journal |vauthors=Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P |title=Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome |journal=Pathol. Biol. |volume=63 |issue=3 |pages=136–43 |date=June 2015 |pmid=25845294 |doi=10.1016/j.patbio.2015.03.001 |url=}}</ref>
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* [[Glomerulopathy]]
* [[Glomerulopathy]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
|-
|-
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |Thromobcytosis
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |Thromobcytosis
! colspan="2" align="left" style="padding: 5px 5px; background: #DCDCDC;" |[[Iron deficiency anemia|Iron deficiency anemia]]
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Iron deficiency anemia|Iron deficiency anemia]]
Inflammatory diseases
Inflammatory diseases


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* [[Paresthesia|Paresthesias]]   
* [[Paresthesia|Paresthesias]]   
* [[Transient ischemic attack|Transient ischemic attacks]]
* [[Transient ischemic attack|Transient ischemic attacks]]
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
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| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | Normal
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
|-
|-
! rowspan="6" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Qualitative Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
! rowspan="6" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Qualitative Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
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* Positive family history
* Positive family history
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | Rare
| align="center" style="background:#F5F5F5;" | Rare
| align="center" style="background:#F5F5F5;" | Normal or ↓
| align="center" style="background:#F5F5F5;" | Normal or ↓
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* Positive family history
* Positive family history
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | Normal/↓
| align="center" style="background:#F5F5F5;" | Normal/↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | Normal or ↓
| align="center" style="background:#F5F5F5;" | Normal or ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki>
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | -
| align="center" style="background:#F5F5F5;" |
| align="center" style="background:#F5F5F5;" | Normal or ↓
| align="center" style="background:#F5F5F5;" | Normal or ↓
| align="center" style="background:#F5F5F5;" | ↑
| align="center" style="background:#F5F5F5;" | ↑
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|-
|-
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Acquired Disorders of [[Platelet]] Function<span name="harr_c115s002s004s002p001"></span><span name="9100808"></span>
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Acquired Disorders of [[Platelet]] Function<span name="harr_c115s002s004s002p001"></span><span name="9100808"></span>
| align="left" style="background:#F5F5F5
| align="left" style="background:#F5F5F5 |
* [[Chronic renal failure pathophysiology|Uremia]]
* [[Chronic renal failure pathophysiology|Uremia]]
* [[Cardiopulmonary bypass]]
* [[Cardiopulmonary bypass]]
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! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Von Willebrand disease|Von Willebrand Disease]]<span name="harr_c115s002s005p001"></span><span name="9100810"></span> <ref name="pmid25196510">{{cite journal |vauthors=Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD |title=Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project |journal=Haemophilia |volume=20 |issue=6 |pages=831–5 |date=November 2014 |pmid=25196510 |pmc=4251588 |doi=10.1111/hae.12503 |url=}}</ref><ref name="pmid25196510">{{cite journal |vauthors=Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD |title=Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project |journal=Haemophilia |volume=20 |issue=6 |pages=831–5 |date=November 2014 |pmid=25196510 |pmc=4251588 |doi=10.1111/hae.12503 |url=}}</ref><ref name="pmid16985174">{{cite journal |vauthors=Goodeve A, Eikenboom J, Castaman G, Rodeghiero F, Federici AB, Batlle J, Meyer D, Mazurier C, Goudemand J, Schneppenheim R, Budde U, Ingerslev J, Habart D, Vorlova Z, Holmberg L, Lethagen S, Pasi J, Hill F, Hashemi Soteh M, Baronciani L, Hallden C, Guilliatt A, Lester W, Peake I |title=Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD) |journal=Blood |volume=109 |issue=1 |pages=112–21 |date=January 2007 |pmid=16985174 |doi=10.1182/blood-2006-05-020784 |url=}}</ref><ref name="pmid9579642">{{cite journal |vauthors=Mammen EF, Comp PC, Gosselin R, Greenberg C, Hoots WK, Kessler CM, Larkin EC, Liles D, Nugent DJ |title=PFA-100 system: a new method for assessment of platelet dysfunction |journal=Semin. Thromb. Hemost. |volume=24 |issue=2 |pages=195–202 |date=1998 |pmid=9579642 |doi=10.1055/s-2007-995840 |url=}}</ref><ref name="pmid258585642">{{cite journal |vauthors=Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J |title=Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=13 |issue=7 |pages=1345–50 |date=July 2015 |pmid=25858564 |pmc=5576173 |doi=10.1111/jth.12964 |url=}}</ref>
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Von Willebrand disease|Von Willebrand Disease]]<span name="harr_c115s002s005p001"></span><span name="9100810"></span> <ref name="pmid25196510">{{cite journal |vauthors=Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD |title=Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project |journal=Haemophilia |volume=20 |issue=6 |pages=831–5 |date=November 2014 |pmid=25196510 |pmc=4251588 |doi=10.1111/hae.12503 |url=}}</ref><ref name="pmid25196510">{{cite journal |vauthors=Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD |title=Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project |journal=Haemophilia |volume=20 |issue=6 |pages=831–5 |date=November 2014 |pmid=25196510 |pmc=4251588 |doi=10.1111/hae.12503 |url=}}</ref><ref name="pmid16985174">{{cite journal |vauthors=Goodeve A, Eikenboom J, Castaman G, Rodeghiero F, Federici AB, Batlle J, Meyer D, Mazurier C, Goudemand J, Schneppenheim R, Budde U, Ingerslev J, Habart D, Vorlova Z, Holmberg L, Lethagen S, Pasi J, Hill F, Hashemi Soteh M, Baronciani L, Hallden C, Guilliatt A, Lester W, Peake I |title=Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD) |journal=Blood |volume=109 |issue=1 |pages=112–21 |date=January 2007 |pmid=16985174 |doi=10.1182/blood-2006-05-020784 |url=}}</ref><ref name="pmid9579642">{{cite journal |vauthors=Mammen EF, Comp PC, Gosselin R, Greenberg C, Hoots WK, Kessler CM, Larkin EC, Liles D, Nugent DJ |title=PFA-100 system: a new method for assessment of platelet dysfunction |journal=Semin. Thromb. Hemost. |volume=24 |issue=2 |pages=195–202 |date=1998 |pmid=9579642 |doi=10.1055/s-2007-995840 |url=}}</ref><ref name="pmid258585642">{{cite journal |vauthors=Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J |title=Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=13 |issue=7 |pages=1345–50 |date=July 2015 |pmid=25858564 |pmc=5576173 |doi=10.1111/jth.12964 |url=}}</ref>
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Revision as of 14:07, 19 October 2018


Bleeding diathesis main page

Overview

Classification

Differential Diagnosis

Platelet disorders
Immune Thrombocytopenic Purpura
Thrombotic Thrombocytopenic Purpura
Hemolytic Uremic Syndrome
Thrombocytosis
Von Willebrand Disease
Coagulation disorders
Fibrinogen deficiency
Prothrombin deficiency
Factor V deficiency
Factor VII deficiency
Factor VIII deficiency
Factor IX deficiency
Factor X deficiency
Factor XI deficiency
Factor XII deficiency
High-molecular-weight kininogen deficiency
Prekallikrein deficiency
Factor XIII deficiency
Hemophilia
Rare diseases
Disseminated Intravascular Coagulation
Vitamin K Deficiency

Different types of Von-Willebrand diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2], Nazia Fuad M.D., Sogand Goudarzi, MD [3]

Synonyms and keywords: Hypocoagulopathy; Bleeding disorders.

Overview

Bleeding diathesis is susceptibility to bleed due to coagulopathy disorders or platelets disorders. These diseases can occur due to a disorder of homeostasis, localized process (tissue injury), or medications. Bleeding diathesis can be resulted from vessel wall injury, platelet disorders, and coagulation factor disorders. Clinical manifestation of bleeding disorders can have a wide range of symptoms from asymptomatic to symptomatic massive and life threatening bleeding. Platelet disorders mostly have skin manifestations such as petechiae, and ecchymoses. In order to find the cause of hypo-coagulopathy; there are established laboratory tests, such as peripheral blood smear, platelet count and platelet function analysis, coagulation factor deficiencies and inhibitors, fibrinolysis tests (eg. D-dimer level), bleeding time (BT), prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time (TT), and reptilase time. In the case of any abnormal bleeding, first line of screening tests are CBC, PT, PTT, BT, and TT.[1]

Classification

Disorders of hemostasis can be classified into two main categories: platelet disorders, and disorders of coagulation. Each category can be further classified as bellow:

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Abnormal hemostasis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

•Patient history-sign & symptom: Deep soft tissue bleeding Mucocutaneus bleeding
•Screen test CBBC-plt-PT&PTT-BT-TT
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hx of deep soft tissue bleeding
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hx of mucocotaneus bleeding
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Coagulopathy
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Plt disorder
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Family history
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal plt count
 
 
Low plt count
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
(+)
•Inherited coagulpathy
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
(-)
•Aquired coagulopathy
 
 
 
 
 
 
Functional Plt disorder
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
↑Plt
•Hemophillia
•VWD
•FactorVIII or IX deficiency
 
↑PT
• Factor VII deficiensy
 
↑PT&↑PTT
•Fibrinogen deficiency
•FactorII deficiency
•FactorV deficiency
•FactorX deficiency
 
 
↑PTT
•Factor inhibitor
•Anti phospholipid A6syndrome
 
↑PT
•Factor inhibitor
•VitK deficiency
•Liver disease
 
↑PT&↑PTT
•Factor inhibit
•DIC
•Liver failure
•late stage of VitK deficincy
 
↑Afibrinogenia
•Heparin inhibitor
•Direct thrombin inhibitor
 
 
 
 
Abnormal solobity
•FactorXIII deficincy
•Cross-linkin inhibitor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Not corrected with mixing with NL plasma
 
 
 
 
 
 
 
 
HX(+)
 
HX(-)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

•Factor inhibitors
•Lupus anti coagulant
•DIC
•Heparin or direct thrombin inhibitors
 
 
 
 
 
 
 
 
Congenital
 
Acquired
 
 
 
 
 

Platelet disorders:

  1. Thrombocytopenia: platelet count less than 150,000 per mm3
  2. Thromobcytosis: platelet count more than 450,000 per mm3
  3. Qualitative Disorders of Platelet function such as Von Willebrand Disease, inherited or acquired functional disorders.

Coagulation disorders

  1. Vessel wall disorders: Endothelial cells are lining entire vessel walls all over the body. Endothelium is an active layer responsible for inflammatory responses, angiogenesis and blood cell interactions. Endothelial cells have a very important role in hemostasis and they are regulating blood fluidity by the balance of antithrombotic/prothrombotic and vasodilatory/vasoconstrictor effects.
  2. Coagulation factor disorders:
  3. Disseminated Intravascular Coagulation
  4. Vitamin K Deficiency
  5. Coagulation Disorders Associated with Liver Failure
  6. Acquired Inhibitors of Coagulation Factors

Differential Diagnosis

Different causes of the bleeding disorders can be differentiated based on their clinical manifestation and laboratory findings.

These features have discussed in the below table:

Category Sub-category Diseases History Clinical manifestation Laboratory testing Comments
Mucosal bleeding Petechia Ecchymoses Menorrhagia Hematoma Hemarthrosis Platelet count Bleeding time (BT) Prothrombin time (PT) Activated partial thromboplastin time (aPTT) Thrombin time (TT)
Platelet disorders Thrombocytopenia Infection-Induced thrombocytopenia[2][3][4]
  • History of prior infection
+ + + + + + Normal Normal Normal
Medications-Induced thrombocytopenia [5][6] + + + + + + Normal Normal Normal Most important part of treatment is discontinuing of the medication.
Heparin-Induced thrombocytopenia[7] + + + + + + Normal Normal For more information click here: Heparin-induced thrombocytopenia.
Immune Thrombocytopenic Purpura (ITP)[8] + + + + + + Normal Normal Normal
Inherited Thrombocytopenia[9][10]
  • Positive family history
+ + + + + + Normal Normal Normal
Thrombotic Thrombocytopenic Purpura (TTP)[11][12] History of: + + + + + + Normal Normal Normal
Hemolytic Uremic Syndrome[13][14] History of: + + + + + + Normal Normal Normal
Thromobcytosis Iron deficiency anemia

Inflammatory diseases

Splenectomy

Essential thrombocytosis

+/- +/- Normal or ↑ Normal Normal Normal
Qualitative Disorders of Platelet Function Inherited Disorders of Platelet Function Glanzmann’s thrombasthenia
  • Positive family history
+ + + + Rare Normal or ↓ Normal Normal Normal
  • AR inheritance
  • Absence of the platelet Gp IIb/IIIa receptor
  • Diminished for GP 2B-3A on flow cytometry
Bernard-Soulier syndrome[15][16]
  • Positive family history
+ + + + Normal/↓ Normal Normal Normal
  • AR inheritance
  • Absence of the platelet Gp Ib-IX-V receptor
  • On PBS: giant platelets
  • Ristocetin - no aggregation
Wiskott-Aldrich syndrome[17][18][19][20]
  • Positive family history
+ + + + Normal or ↓ Normal Normal Normal
  • Anti-WASP antibody can be used to detect presence or absence of WAS protein
  • In Wiskott–Aldrich syndrome, the platelets are small and do not function properly. They are removed by the spleen, which leads to low platelet counts.
Platelet storage pool disorder (SPD): + + + + Normal or ↓ Normal Normal Normal
  • AD inheritance
  • Abnormalities of platelet granule formation
Acquired Disorders of Platelet Function + + + + +/- +/- Normal/↓ Normal Normal Normal
Von Willebrand Disease [21][21][22][23][24] + + + + +/- +/- Normal Normal See the table below for the details about different types.
Vessel wall disorders Metabolic and Inflammatory Disorders
  • History of the underlying disease.
+ + +/- - - Normal ↑/Normal Normal Normal Normal -
Inherited Disorders of the Vessel Wall
  • Positive family history
- + + +/- - - Normal ↑/Normal Normal Normal Normal -
Coagulation factor disorders[25][26][27][27][28][29][30][31][32][33][34] Fibrinogen deficiency[35] Different types of the fibrinogen disorders: - - + + +/- + Normal
  • Impaired fibrin cross linking or clot dissolution.
  • The severity of bleeding in patients with fibrinogen disorders can be mild or severe, with higher bleeding risk in those with afibrinogenemia or lower levels of functional fibrinogen. The age of onset is also variable, with earlier onset in those with more severe deficiency.
Prothrombin deficiency + + + + + Normal Normal -
Factor V deficiency _ + + + + Normal Normal The severity of bleeding is only partly related to the degree of factor V deficiency. Some patients with undetectable plasma levels of factor V experience only relatively mild bleeding.
Factor VII deficiency + + + Normal Normal Normal Thrombosis occurs in inherited factor VII deficiency most cases are associated with the administration of factor VII replacement therapy
Factor X deficiency
  • Prolonged bleeding following circumcision
+ + + + + Normal Normal Normal -
Factor XII deficiency
  • Majority,asymptomatic
  • Recurrent miscarriages
  • Painful leg ulcers
_ _ _ _ _ Normal Normal Normal Normal
High molecular weight kininogen (HMWK) deficiency
  • Possibility of positive family history of bleeding
_ _ _ _ _ Normal Normal Normal Normal
Prekallikrein deficiency
  • Possibility of positive family history of bleeding
_ _ _ _ _ Normal Normal Normal Normal
Factor XIII deficiency Types:
  • Sub unit A mutation disease (more common)
  • Sub unit B mutation disease
  • Possibility of positive family history of bleeding
-/+ -/+ -/+ -/+ -/+ -/+ Normal Normal Normal/↑ Normal Normal
  • Impaired fibrin cross linking or clot dissolution
  • The severity of factor XIII deficiency bleeds can be different in different patients
Hemophilia[36][37][38][39][40][41] Type A deficiency - - - + + + Normal Normal Normal Normal -
Type B deficiency - - - + + + Normal Normal Normal Normal -
Type C deficiency
  • Family history
  • Bleeding after surgery or injury
- - - + Rare Rare Normal Normal Normal Normal -
Rare diseases Disseminated Intravascular Coagulation[42] + + + + + + Normal -
Vitamin K Deficiency[43] + - + + + + Normal Normal or mildly prolonged Normal -

Different types of Von-Willebrand diseases can be differentiated from each other based on the following table:[44]

Type of VWD Type of factor deficiency Prevalence Inheritance pattern Clinical manifestations VWF activity RIPA Factor VIII
Type 1 Quantitative/ partial 60-70% AD
  • Bleeding severity mild to severe
Type 2 2A[45] Qualitative 10% AD/AR N or ↓
2B Qualitative 5% AD N or ↓
2M Qualitative <1% AD/AR N or ↓
2N Qualitative <1% AR N N
Type 3 Complete deficiency 1-2% AR Absent Low, 1-10%

For more information on Von Willebrand disease, click here.

References

  1. Posan E, McBane RD, Grill DE, Motsko CL, Nichols WL (September 2003). "Comparison of PFA-100 testing and bleeding time for detecting platelet hypofunction and von Willebrand disease in clinical practice". Thromb. Haemost. 90 (3): 483–90. doi:10.1160/TH03-02-0111. PMID 12958618.
  2. Neunert C, Lim W, Crowther M, Cohen A, Solberg L, Crowther MA (April 2011). "The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia". Blood. 117 (16): 4190–207. doi:10.1182/blood-2010-08-302984. PMID 21325604.
  3. Karimi O, Goorhuis A, Schinkel J, Codrington J, Vreden S, Vermaat JS, Stijnis C, Grobusch MP (March 2016). "Thrombocytopenia and subcutaneous bleedings in a patient with Zika virus infection". Lancet. 387 (10022): 939–940. doi:10.1016/S0140-6736(16)00502-X. PMID 26906627. Vancouver style error: initials (help)
  4. Zammarchi L, Stella G, Mantella A, Bartolozzi D, Tappe D, Günther S, Oestereich L, Cadar D, Muñoz-Fontela C, Bartoloni A, Schmidt-Chanasit J (February 2015). "Zika virus infections imported to Italy: clinical, immunological and virological findings, and public health implications". J. Clin. Virol. 63: 32–5. doi:10.1016/j.jcv.2014.12.005. PMID 25600600.
  5. Kam T, Alexander M (October 2014). "Drug-induced immune thrombocytopenia". J Pharm Pract. 27 (5): 430–9. doi:10.1177/0897190014546099. PMID 25134884.
  6. Seco-Melantuche R, Delgado-Sánchez O, Álvarez-Arroyo L (2013). "[Incidence of drug-induced thrombocytopenia in hospitalized patients]". Farm Hosp (in Spanish; Castilian). 37 (1): 27–34. doi:10.7399/FH.2013.37.1.42. PMID 23461497.
  7. Warkentin TE, Safyan EL, Linkins LA (January 2015). "Heparin-induced thrombocytopenia presenting as bilateral adrenal hemorrhages". N. Engl. J. Med. 372 (5): 492–4. doi:10.1056/NEJMc1414161. PMID 25629757.
  8. Wright JF, Blanchette VS, Wang H, Arya N, Petric M, Semple JW, Chia WK, Freedman J (October 1996). "Characterization of platelet-reactive antibodies in children with varicella-associated acute immune thrombocytopenic purpura (ITP)". Br. J. Haematol. 95 (1): 145–52. PMID 8857953.
  9. Johnson B, Fletcher SJ, Morgan NV (September 2016). "Inherited thrombocytopenia: novel insights into megakaryocyte maturation, proplatelet formation and platelet lifespan". Platelets. 27 (6): 519–25. doi:10.3109/09537104.2016.1148806. PMC 5000870. PMID 27025194.
  10. Wang Q, Cao L, Sheng G, Shen H, Ling J, Xie J, Ma Z, Yin J, Wang Z, Yu Z, Chen S, Zhao Y, Ruan C, Xia L, Jiang M (August 2018). "Application of High-Throughput Sequencing in the Diagnosis of Inherited Thrombocytopenia". Clin. Appl. Thromb. Hemost.: 1076029618790696. doi:10.1177/1076029618790696. PMID 30103613.
  11. Knöbl P (2018). "Thrombotic thrombocytopenic purpura". Memo. 11 (3): 220–226. doi:10.1007/s12254-018-0429-6. PMID 30220931.
  12. Mannucci PM, Cugno M (November 2015). "The complex differential diagnosis between thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: Laboratory weapons and their impact on treatment choice and monitoring". Thromb. Res. 136 (5): 851–4. doi:10.1016/j.thromres.2015.09.007. PMID 26386489.
  13. Webster K, Schnitzler E (2014). "Hemolytic uremic syndrome". Handb Clin Neurol. 120: 1113–23. doi:10.1016/B978-0-7020-4087-0.00075-9. PMID 24365375.
  14. Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P (June 2015). "Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome". Pathol. Biol. 63 (3): 136–43. doi:10.1016/j.patbio.2015.03.001. PMID 25845294.
  15. Dupuis A, Gachet C (September 2018). "Inherited platelet disorders : Management of the bleeding risk". Transfus Clin Biol. 25 (3): 228–235. doi:10.1016/j.tracli.2018.07.003. PMID 30077511.
  16. Andres O, Henning K, Strauß G, Pflug A, Manukjan G, Schulze H (June 2018). "Diagnosis of platelet function disorders: A standardized, rational, and modular flow cytometric approach". Platelets. 29 (4): 347–356. doi:10.1080/09537104.2017.1386297. PMID 29227167.
  17. Wang YQ, Cui YX, Feng J (January 2013). "[Clinical phenotype and gene diagnostic analysis of Omenn syndrome]". Zhonghua Er Ke Za Zhi (in Chinese). 51 (1): 64–8. PMID 23527934.
  18. Patil RB, Shanmukhaiah C, Jijina F, Bamborde S, Wasekar N, Toshniwal M, Mohite A, Patil V (2016). "Wiskott-Aldrich Syndrome Presenting with JMML-Like Blood Picture and Normal Sized Platelets". Case Rep Hematol. 2016: 8230786. doi:10.1155/2016/8230786. PMC 4906177. PMID 27340577.
  19. Kaneko R, Yamamoto S, Okamoto N, Akiyama K, Matsuno R, Toyama D, Hoshino A, Imai K, Isoyama K (2018). "Wiskott-Aldrich syndrome that was initially diagnosed as immune thrombocytopenic purpura secondary to a cytomegalovirus infection". SAGE Open Med Case Rep. 6: 2050313X17753788. doi:10.1177/2050313X17753788. PMC 5768273. PMID 29348920.
  20. Ozcan E, Notarangelo LD, Geha RS (December 2008). "Primary immune deficiencies with aberrant IgE production". J. Allergy Clin. Immunol. 122 (6): 1054–62, quiz 1063–4. doi:10.1016/j.jaci.2008.10.023. PMID 19084106.
  21. 21.0 21.1 Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD (November 2014). "Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project". Haemophilia. 20 (6): 831–5. doi:10.1111/hae.12503. PMC 4251588. PMID 25196510.
  22. Goodeve A, Eikenboom J, Castaman G, Rodeghiero F, Federici AB, Batlle J, Meyer D, Mazurier C, Goudemand J, Schneppenheim R, Budde U, Ingerslev J, Habart D, Vorlova Z, Holmberg L, Lethagen S, Pasi J, Hill F, Hashemi Soteh M, Baronciani L, Hallden C, Guilliatt A, Lester W, Peake I (January 2007). "Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD)". Blood. 109 (1): 112–21. doi:10.1182/blood-2006-05-020784. PMID 16985174.
  23. Mammen EF, Comp PC, Gosselin R, Greenberg C, Hoots WK, Kessler CM, Larkin EC, Liles D, Nugent DJ (1998). "PFA-100 system: a new method for assessment of platelet dysfunction". Semin. Thromb. Hemost. 24 (2): 195–202. doi:10.1055/s-2007-995840. PMID 9579642.
  24. Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J (July 2015). "Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH". J. Thromb. Haemost. 13 (7): 1345–50. doi:10.1111/jth.12964. PMC 5576173. PMID 25858564.
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