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Article ; Online: Acquired factor V and factor X Deficiency coexisting with acquired dysfibrinogenaemia in a patient with light chain myeloma.

Jeffrey, Susan / Hamilton, Craig / Ames, Paul R J

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis

2024 Volume 35, Issue 3, Page(s) 139–140

Abstract: An elderly woman with light chain myeloma presented with prolonged epistaxis and extensive cutaneous haematomas: her kappa/lambda ratio was high at 395, her coagulation screen, thrombin and reptilase times were abnormal, her FV and FX were in the low ... ...

Abstract	An elderly woman with light chain myeloma presented with prolonged epistaxis and extensive cutaneous haematomas: her kappa/lambda ratio was high at 395, her coagulation screen, thrombin and reptilase times were abnormal, her FV and FX were in the low range in the absence of specific inhibitors, her Clauss fibrinogen was low at 0.95 g/l but antigenic FNG was 1.58 g/l. The patient denied treatment and died of progressive renal failure. We wish to describe the unusual association of FX and FV deficiency co-existing with an acquired dysfibrinogenaemia.
MeSH term(s)	Aged ; Female ; Humans ; Afibrinogenemia/complications ; Factor V ; Factor X Deficiency ; Fibrinogen ; Multiple Myeloma/complications
Chemical Substances	Factor V (9001-24-5) ; Fibrinogen (9001-32-5)
Language	English
Publishing date	2024-02-07
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	1033551-1
ISSN	1473-5733 ; 0957-5235
ISSN (online)	1473-5733
ISSN	0957-5235
DOI	10.1097/MBC.0000000000001280
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Article ; Online: Efficacy of extended microthrombolysis in an elderly lady with purpura fulminans.

Ames, Paul R J / Nale, Krsty / Malone, Colin

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis

2023 Volume 34, Issue 4, Page(s) 247–249

Abstract: Purpura fulminans is a thrombotic emergency affecting small vessels of skin and of internal organs that may rapidly progress into necrotizing fasciitis, critical limb ischaemia and multiorgan failure; it often develops during an infection or as a ... ...

Abstract	Purpura fulminans is a thrombotic emergency affecting small vessels of skin and of internal organs that may rapidly progress into necrotizing fasciitis, critical limb ischaemia and multiorgan failure; it often develops during an infection or as a postinfective 'autoimmune' disorder. Although supportive care and hydration are important, anticoagulation ought to be started to prevent further occlusions alongside blood products according to need. Herein, we describe the case of an elderly woman who received extended intravenous low-dose recombinant tissue plasminogen activator at the onset of purpura fulminans that salvaged her skin and prevented the development of multiorgan failure.
MeSH term(s)	Humans ; Female ; Aged ; Purpura Fulminans/drug therapy ; Purpura Fulminans/etiology ; Tissue Plasminogen Activator/therapeutic use ; Thrombosis ; Multiple Organ Failure
Chemical Substances	Tissue Plasminogen Activator (EC 3.4.21.68)
Language	English
Publishing date	2023-03-02
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	1033551-1
ISSN	1473-5733 ; 0957-5235
ISSN (online)	1473-5733
ISSN	0957-5235
DOI	10.1097/MBC.0000000000001209
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Zs.A 2920: Show issues

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Article ; Online: Relevance of vein wall thickness in Behcet's disease: A systematic review and meta-analysis.

Merashli, Mira / Bucci, Tommaso / Delgado-Alves, Jose / Ames, Paul R J

Autoimmunity reviews

2023 Volume 23, Issue 2, Page(s) 103487

Abstract: Objectives: To perform a meta-analysis on articles evaluating the common femoral vein wall thickness (VWT) in Behcet's disease and its possible clinical, laboratory and treatment correlates (BD).: Methods: Systematic search of EMBASE and PubMed ... ...

Abstract	Objectives: To perform a meta-analysis on articles evaluating the common femoral vein wall thickness (VWT) in Behcet's disease and its possible clinical, laboratory and treatment correlates (BD). Methods: Systematic search of EMBASE and PubMed databases from inception to October 2023; we employed random effect meta-analyses for continuous outcomes. Results: The meta-analysis included 9 case-control and 1 cohort study: the VWT was greater in BD (n = 650) than in controls (n = 396) (p < 0.0001) with wide heterogeneity (I Conclusion: VWT is greater in BD than controls: age, male gender, disease duration and smoking relate to VWT that was greater in BD patients with a history of thrombotic/vascular disease. Prospective studies are required to assess whether VWT may be considered a vascular marker of disease activity.
Language	English
Publishing date	2023-11-29
Publishing country	Netherlands
Document type	Journal Article ; Review
ZDB-ID	2144145-5
ISSN	1873-0183 ; 1568-9972
ISSN (online)	1873-0183
ISSN	1568-9972
DOI	10.1016/j.autrev.2023.103487
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Article ; Online: Risk of death, thrombotic and hemorrhagic events in anticoagulated patients with atrial fibrillation and systemic autoimmune diseases: an analysis from a global federated dataset.

Bucci, Tommaso / Cardamone, Chiara / Triggiani, Massimo / Ames, Paul R J / Lip, Gregory Y H

Clinical research in cardiology : official journal of the German Cardiac Society

2024

Abstract: Background: Growing evidence showing that systemic autoimmune diseases (SADs) are associated with a high risk of atrial fibrillation (AF). However, the impact of SAD on the clinical course of AF patients is largely unknown.: Methods: Retrospective ... ...

Abstract	Background: Growing evidence showing that systemic autoimmune diseases (SADs) are associated with a high risk of atrial fibrillation (AF). However, the impact of SAD on the clinical course of AF patients is largely unknown. Methods: Retrospective cohort study within a federated healthcare network (TriNetX). Using ICD codes, AF patients on anticoagulant therapy were categorized according to the presence of SAD (M32: Systemic Lupus Erythematosus (SLE); M33: Dermato-polymyositis (DMP); M34: Systemic Sclerosis (SSc); M35: Sjogren syndrome). The primary outcomes were the 5-year risks of (1) all-cause death, (2) thrombotic events (ischemic stroke, acute myocardial infarction, deep vein thrombosis, and pulmonary embolism), and (3) bleeding (intracranial (ICH) and gastrointestinal (GI)). Secondary outcomes were each component of the primary outcomes. Cox regression analysis after propensity score matching (PSM) was used to estimate hazard ratio (HR) and 95% confidence interval (95%CI). Results: We identified 16,098 AF patients with SAD (68.2 ± 13.4 years; 71.0% female) and 828,772 AF controls (70.7 ± 12.9 years, 41.1% females). After PSM, AF patients with SAD were associated with a higher risk of all-cause death (HR 1.13, 95%CI 1.09-1.71), thrombotic events (HR 1.37, 95%CI 1.32-1.43), and hemorrhagic events (HR 1.41, 95%CI 1.33-1.50) compared to AF controls without SAD. The highest risk of all-cause death and GI bleeding was associated with SSc, while the highest risk of thrombotic events and ICH was associated with SLE. Conclusion: AF patients with SAD are associated with a high risk of all-cause death, thrombotic, and hemorrhagic events. These patients merit careful follow-up and integrated care management to improve their prognosis.
Language	English
Publishing date	2024-03-06
Publishing country	Germany
Document type	Journal Article
ZDB-ID	2213295-8
ISSN	1861-0692 ; 1861-0684
ISSN (online)	1861-0692
ISSN	1861-0684
DOI	10.1007/s00392-024-02426-1
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Article ; Online: Bortezomib and rituximab in multiply relapsed primary warm autoimmune hemolytic anemia.

Ames, Paul R J / Jeffrey, Susan

Annals of hematology

2020 Volume 100, Issue 9, Page(s) 2415–2416

MeSH term(s)	Anemia, Hemolytic, Autoimmune/drug therapy ; Antibodies, Monoclonal, Murine-Derived ; Bortezomib ; Humans ; Rituximab
Chemical Substances	Antibodies, Monoclonal, Murine-Derived ; Rituximab (4F4X42SYQ6) ; Bortezomib (69G8BD63PP)
Language	English
Publishing date	2020-06-17
Publishing country	Germany
Document type	Letter ; Comment
ZDB-ID	1064950-5
ISSN	1432-0584 ; 0939-5555 ; 0945-8077
ISSN (online)	1432-0584
ISSN	0939-5555 ; 0945-8077
DOI	10.1007/s00277-020-04135-x
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Article ; Online: Subclinical atherosclerosis in Behcet's disease and its inverse relation to azathioprine use: an updated meta-analysis.

Merashli, Mira / Bucci, Tommaso / Arcaro, Alessia / Gentile, Fabrizio / Ames, Paul R J

Clinical and experimental medicine

2023 Volume 23, Issue 7, Page(s) 3431–3442

Abstract: To evaluate the intima media thickness of carotid arteries (IMT) and its clinical, laboratory and treatment correlates in Behcet's disease (BD). Systematic search of EMBASE and PubMed databases from January 2016 to October 2022; we employed random effect ...

Abstract	To evaluate the intima media thickness of carotid arteries (IMT) and its clinical, laboratory and treatment correlates in Behcet's disease (BD). Systematic search of EMBASE and PubMed databases from January 2016 to October 2022; we employed random effect meta-analyses for continuous outcomes and Peto's odds ratio for rare events. The meta-analysis included 36 case control studies: the IMT was greater in BD (n = 1103) than in controls (n = 832) (p < 0.0001) with wide heterogeneity (I
MeSH term(s)	Humans ; Male ; Behcet Syndrome/complications ; Behcet Syndrome/drug therapy ; Carotid Intima-Media Thickness ; Azathioprine/therapeutic use ; Atherosclerosis ; Plaque, Atherosclerotic ; Risk Factors
Chemical Substances	Azathioprine (MRK240IY2L)
Language	English
Publishing date	2023-05-11
Publishing country	Italy
Document type	Meta-Analysis ; Journal Article
ZDB-ID	2053018-3
ISSN	1591-9528 ; 1591-8890
ISSN (online)	1591-9528
ISSN	1591-8890
DOI	10.1007/s10238-023-01084-3
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Zs.A 5481: Show issues

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Article ; Online: Thrombocytopaenia in antiphospholipid syndrome: a free radical perspective.

Ames, Paul R J / Bucci, Tommaso / Merashli, Mira / Arcaro, Alessia / Gentile, Fabrizio

Rheumatology (Oxford, England)

2022 Volume 62, Issue 6, Page(s) 2070–2075

Abstract: Thrombosis associated with thrombocytopaenia is an apparent paradox that is present across a wide spectrum of disorders. While thrombocytopaenia has been a controversial clinical classification criterion for APS, as initial reports failed to demonstrate ... ...

Abstract	Thrombosis associated with thrombocytopaenia is an apparent paradox that is present across a wide spectrum of disorders. While thrombocytopaenia has been a controversial clinical classification criterion for APS, as initial reports failed to demonstrate a relation between low platelet count with other clinical or laboratory manifestations of the syndrome, recent data highlight the association between mild-moderate thrombocytopaenia and the risk of thrombosis. Although aPL antibodies may induce platelet activation in vitro, additional stimuli may contribute to their activation in vivo, among which are reactive oxygen and nitrogen species and lipid peroxidation products, which are elevated in patients with APS; an excess of the same stimuli may induce megakaryocyte and platelet apoptosis that leads to decreased platelet production and increased destruction, resulting ultimately in thrombocytopaenia. Herein we provide a novel plausible framework involving free radicals that could add to the understanding of the thrombocytopaenia-thrombosis paradox in APS.
MeSH term(s)	Humans ; Antiphospholipid Syndrome/complications ; Antibodies, Antiphospholipid ; Thrombosis ; Leukopenia ; Free Radicals ; Thrombocytopenia/complications
Chemical Substances	Antibodies, Antiphospholipid ; Free Radicals
Language	English
Publishing date	2022-11-18
Publishing country	England
Document type	Journal Article
ZDB-ID	1464822-2
ISSN	1462-0332 ; 1462-0324
ISSN (online)	1462-0332
ISSN	1462-0324
DOI	10.1093/rheumatology/keac650
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Zs.MO 497: Show issues

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Article ; Online: Relevance of Plasma Homocysteine and Methylenetetrahydrofolate Reductase 677TT Genotype in Sickle Cell Disease: A Systematic Review and Meta-Analysis.

Ames, Paul R J / Arcaro, Alessia / Caruso, Matilde / Graf, Maria / Marottoli, Vincenzo / Gentile, Fabrizio

International journal of molecular sciences

2022 Volume 23, Issue 23

Abstract: We evaluated the relevance of plasma homocysteine (HC) and the TT genotype of the methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism (rs1801133) in sickle cell disease (SCD) and associated vaso-occlusive crisis (VOC) and ischemic stroke (IS). ...

Abstract	We evaluated the relevance of plasma homocysteine (HC) and the TT genotype of the methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism (rs1801133) in sickle cell disease (SCD) and associated vaso-occlusive crisis (VOC) and ischemic stroke (IS). We identified in Embase and Medline 22 studies on plasma HC and 22 on MTHFR genotypes. Due to age-related HC differences, adult and paediatric SCD were separated: 879 adult SCD and 834 controls (CTR) yielded a neutral effect size; 427 paediatric SCD and 625 CTR favoured SCD (p = 0.001) with wide heterogeneity (I2 = 95.5%) and were sub-grouped by country: six studies (Dutch Antilles n = 1, USA n = 5) yielded a neutral effect size, four (India n = 1, Arab countries n = 3) favoured SCD (p < 0.0001). Moreover, 249 SCD in VOC and 419 out of VOC yielded a neutral effect size. The pooled prevalence of the MTHFR TT genotype in 267 SCD equalled that of 1199 CTR (4.26% vs. 2.86%, p = 0.45), and in 84 SCD with IS equalled that of 86 without IS (5.9% vs. 3.7%, p = 0.47); removal of one paediatric study yielded a significant effect size (p = 0.006). Plasma HC in paediatric SCD from Middle East and India was higher, possibly due to vitamin deficiencies. Despite its low prevalence in SCD, the MTHFR TT genotype relates to adult IS.
MeSH term(s)	Adult ; Child ; Humans ; Anemia, Sickle Cell/diagnosis ; Anemia, Sickle Cell/genetics ; Genotype ; Homocysteine/blood ; Methylenetetrahydrofolate Reductase (NADPH2)/genetics ; Polymorphism, Genetic
Chemical Substances	Homocysteine (0LVT1QZ0BA) ; Methylenetetrahydrofolate Reductase (NADPH2) (EC 1.5.1.20)
Language	English
Publishing date	2022-11-24
Publishing country	Switzerland
Document type	Meta-Analysis ; Systematic Review ; Journal Article ; Review
ZDB-ID	2019364-6
ISSN	1422-0067 ; 1422-0067 ; 1661-6596
ISSN (online)	1422-0067
ISSN	1422-0067 ; 1661-6596
DOI	10.3390/ijms232314641
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Article ; Online: Intima media thickness of carotid arteries in familial Mediterranean fever: a systematic review and meta-analysis.

Merashli, Mira / Bucci, Tommaso / Pastori, Daniele / Pignatelli, Pasquale / Ames, Paul R J

Clinical rheumatology

2022 Volume 41, Issue 12, Page(s) 3769–3776

Abstract: Aim: To perform a systematic review and meta-analysis of studies reporting data on atherosclerosis and inflammatory markers in familial Mediterranean fever (FMF).: Methods: EMBASE and PubMed databases were screened according to PRISMA guidelines from ...

Abstract	Aim: To perform a systematic review and meta-analysis of studies reporting data on atherosclerosis and inflammatory markers in familial Mediterranean fever (FMF). Methods: EMBASE and PubMed databases were screened according to PRISMA guidelines from inception to January 2022 for articles reporting measurements of the intima media thickness (IMT) of carotid arteries and eventually carotid plaques; random effect meta-analyses for continuous outcomes and Peto's odds ratio for rare events were employed. Results: The screening and selection search strategy yielded 18 case controls studies (16 full papers and 2 abstracts); the IMT was greater in FMF (n = 1112) than in controls (n = 901) (p < 0.0001) with wide heterogeneity (I Conclusion: FMF is characterised by premature atherosclerosis expressed as a thicker intima media and a greater prevalence of carotid plaques, partially related to the C-reactive protein, as expected by the autoinflammatory nature of FMF. Key Points • Familial Mediterranean fever is characterised by premature atherosclerosis. • C-reactive protein relates to intima media thickness in keeping with the autoinflammatory nature Familial Mediterranean fever. • Targeting the inter-critical low-grade inflammation may be relevant to minimise the additional cardiovascular risk posed by premature atherosclerosis.
MeSH term(s)	Male ; Humans ; Female ; Carotid Intima-Media Thickness ; Familial Mediterranean Fever/complications ; C-Reactive Protein ; Carotid Arteries/diagnostic imaging ; Atherosclerosis/diagnostic imaging ; Plaque, Atherosclerotic
Chemical Substances	C-Reactive Protein (9007-41-4)
Language	English
Publishing date	2022-08-06
Publishing country	Germany
Document type	Meta-Analysis ; Systematic Review ; Journal Article
ZDB-ID	604755-5
ISSN	1434-9949 ; 0770-3198
ISSN (online)	1434-9949
ISSN	0770-3198
DOI	10.1007/s10067-022-06326-5
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Article ; Online: A Meta-Analysis of Plasma Homocysteine in Buerger's Disease.

Merashli, Mira / Bucci, Tommaso / Pastori, Daniele / Pignatelli, Pasquale / Arcaro, Alessia / Gentile, Fabrizio / Marottoli, Vincenzo / Ames, Paul R J

Thrombosis and haemostasis

2022 Volume 122, Issue 7, Page(s) 1243–1246

MeSH term(s)	Homocysteine ; Humans ; Thromboangiitis Obliterans/diagnosis
Chemical Substances	Homocysteine (0LVT1QZ0BA)
Language	English
Publishing date	2022-01-20
Publishing country	Germany
Document type	Meta-Analysis ; Letter
ZDB-ID	518294-3
ISSN	2567-689X ; 0340-6245
ISSN (online)	2567-689X
ISSN	0340-6245
DOI	10.1055/s-0041-1742165
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