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Published in: Autoimmunity Highlights 1/2019

Open Access 01-12-2019 | Acute Pancreatitis | Case Report

Antiphospholipid syndrome: a case report with an unusual wide spectrum of clinical manifestations

Authors: Carmela Mazzoccoli, Domenico Comitangelo, Alessia D’Introno, Valeria Mastropierro, Carlo Sabbà, Antonio Perrone

Published in: Autoimmunity Highlights | Issue 1/2019

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Abstract

Background

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis, and the detection of circulating antiphospholipid antibodies. The classification criteria for definite APS are actually met when at least one clinical criterion (thrombosis or pregnancy morbidity) is present in association of one laboratory criterion (LAC, aCL antibody or aβ2GPI antibody present on two or more occasions, at least 12 weeks a part), and thrombosis should be confirmed by objective validated criteria. The average age of primary APS patients has been reported to be about 35–40 years and the disease is more common in women than in men.

Case presentation

In this report, we described a rare case of an adult male who presented over a period of 9 years with a wide spectrum of clinical manifestations involving different organs that were not initially diagnosed as APS. Dizziness and syncope were his first clinical symptoms, and a non-bacterial thrombotic endocarditis (NBTE) involving the mitral valve was at first diagnosed. Subsequently, the patient also presented with generalized seizures and subsequent head injury. When the patient was admitted to our clinic with bilateral epistaxis and fever, thrombocytopenia was revealed. Moreover, laboratory examinations showed acute pancreatitis with an increase of levels of inflammation markers.

Conclusion

Based on the patient’s medical history and all the examination results, it was possible to make a diagnosis of primary APS and, starting from diagnosis of thrombocytopenia, we were allowed to conclude that all of manifestation were epi-phenomena of a unique clinical entity, rather than unrelated diseases. Though APS is one of the most common thrombocytophilias, unfortunately, it is not recognized often enough. The lack of prevention in undiagnosed patients may cause severe complications which can in turn result in the death of those patients.
Literature
1.
go back to reference Khamashta MA, Bertolaccini Ml, Hughes GR. Antiphospholipid (Hughes) syndrome. Autoimmunity. 2004;37:309–12.CrossRefPubMed Khamashta MA, Bertolaccini Ml, Hughes GR. Antiphospholipid (Hughes) syndrome. Autoimmunity. 2004;37:309–12.CrossRefPubMed
4.
go back to reference Ahluwalia Jasmina, Sreedharanunni Sreejesh. The laboratory diagnosis of the antiphospholipid syndrome. Indian J Hematol Blood Transfus. 2017;33(1):8–14.CrossRefPubMed Ahluwalia Jasmina, Sreedharanunni Sreejesh. The laboratory diagnosis of the antiphospholipid syndrome. Indian J Hematol Blood Transfus. 2017;33(1):8–14.CrossRefPubMed
5.
go back to reference Devreese KMJ, Ortel TL, Pengo V, De Laat B. Laboratory criteria for antiphospholipid syndrome: communication from the Ssc of the Isth. J Thromb Haemos. 2018;16:809–13.CrossRef Devreese KMJ, Ortel TL, Pengo V, De Laat B. Laboratory criteria for antiphospholipid syndrome: communication from the Ssc of the Isth. J Thromb Haemos. 2018;16:809–13.CrossRef
7.
10.
go back to reference Atanassova Penka A. antiphospholipid syndrome and vascular ischemic (occlusive) diseases: an overview. YonseiMed J. 2007;48(6):901–26.CrossRef Atanassova Penka A. antiphospholipid syndrome and vascular ischemic (occlusive) diseases: an overview. YonseiMed J. 2007;48(6):901–26.CrossRef
11.
go back to reference Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthr Rheumatol. 2002;46:1019–27. https://doi.org/10.1002/art.10187.CrossRef Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthr Rheumatol. 2002;46:1019–27. https://​doi.​org/​10.​1002/​art.​10187.CrossRef
13.
go back to reference Ibrahim AM, Siddique MS. Libman Sacks Endocarditis. StatPearls. Treasure Island: StatPearls Publishing; 2019. Ibrahim AM, Siddique MS. Libman Sacks Endocarditis. StatPearls. Treasure Island: StatPearls Publishing; 2019.
19.
go back to reference Diz-Kucukkaya R, Hacihanefioglu A, Yenerel M, et al. Antiphospholipid antibodies and antiphospholipid syndrome in patients presenting with immune thrombocytopenic purpura: a prospective cohort study. Blood. 2001;98:1760–4.CrossRefPubMed Diz-Kucukkaya R, Hacihanefioglu A, Yenerel M, et al. Antiphospholipid antibodies and antiphospholipid syndrome in patients presenting with immune thrombocytopenic purpura: a prospective cohort study. Blood. 2001;98:1760–4.CrossRefPubMed
20.
21.
go back to reference Uthman I, Khamashta M. The abdominal manifestations of the antiphospholipid syndrome. Rheumatology (Oxford). 2007;46(11):1641–7 (Epub 2007 Jul 17).CrossRef Uthman I, Khamashta M. The abdominal manifestations of the antiphospholipid syndrome. Rheumatology (Oxford). 2007;46(11):1641–7 (Epub 2007 Jul 17).CrossRef
22.
go back to reference Asherson RA, Cervera R. Unusual manifestations of the antiphospholipid syndrome. Clin Rev Allergy Immunol. 2003;25(1):61–78.CrossRefPubMed Asherson RA, Cervera R. Unusual manifestations of the antiphospholipid syndrome. Clin Rev Allergy Immunol. 2003;25(1):61–78.CrossRefPubMed
24.
go back to reference Cervera R, Asherson RA. Antiphospholipid syndrome associated with infections: clinical and microbiological characteristics. Immunobiology. 2005;210:735–41.CrossRefPubMed Cervera R, Asherson RA. Antiphospholipid syndrome associated with infections: clinical and microbiological characteristics. Immunobiology. 2005;210:735–41.CrossRefPubMed
Metadata
Title
Antiphospholipid syndrome: a case report with an unusual wide spectrum of clinical manifestations
Authors
Carmela Mazzoccoli
Domenico Comitangelo
Alessia D’Introno
Valeria Mastropierro
Carlo Sabbà
Antonio Perrone
Publication date
01-12-2019
Publisher
BioMed Central
Published in
Autoimmunity Highlights / Issue 1/2019
Print ISSN: 2038-0305
Electronic ISSN: 2038-3274
DOI
https://doi.org/10.1186/s13317-019-0119-3

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