Gingival bleeding, epistaxis and haematoma three days after gastroenteritis: the haemorrhagic lupus anticoagulant syndrome

Schmugge, Markus; Tölle, Sandra; Marbet, G A; Laroche, P.; Meili, Esther

doi:10.1007/pl00008415

Cited by 29 publications

(21 citation statements)

References 7 publications

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“…In children, they tend to appear transiently after viral infections and, in contrast to what is seen in adults, they are usually not associated with other underlying disorders but can occur in conjunction with SLE or malignancy [13,14]. Although rare, there are reports on children who present with bleeding related to circulating antibodies [5,6,19,21,31,[8][9][10][11][12]26,32]. The presentations described vary from all forms of mucosal and non-mucosal bleeding -bloody diarrhea, epistaxis, menorrhagia, purpura, macroscopic haematuria, bruising or spontaneous haematoma formation.…”

Section: Discussionmentioning

confidence: 99%

“…The presentations described vary from all forms of mucosal and non-mucosal bleeding -bloody diarrhea, epistaxis, menorrhagia, purpura, macroscopic haematuria, bruising or spontaneous haematoma formation. Sporadic case reports suggest that viral illness may be an etiological factor [30,31]. Adenovirus has been implicated in most of the reported cases [19], but other viruses such as varicella, cytomegalovirus and epstein-barr virus have been associated.…”

Section: Discussionmentioning

confidence: 99%

“…In addition there have been cases with acquired hypoprothrombinaemia and bleeding, most often in combination with LA [5,6,19,21,31,[8][9][10][11][12]26,32]. Haemorrhagic LA syndrome is rare, less than 30 patients are described in the medical literature, and it has been attributed to increased clearance of prothrombin caused by non-neutralizing antiprothrombin antibodies.…”

Section: Introductionmentioning

confidence: 99%

“…Haemorrhagic LA syndrome is rare, less than 30 patients are described in the medical literature, and it has been attributed to increased clearance of prothrombin caused by non-neutralizing antiprothrombin antibodies. Their signs and symptoms have varied from mild bleeding such as bruising and haematoma to severe gum bleeding, epistaxis and in two cases haemarthrosis [5,6,19,21,31,[8][9][10][11][12]26,32].…”

Section: Introductionmentioning

confidence: 99%

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Lupus anticoagulants in two children—bleeding due to nonphospholipid-dependent antiprothrombin antibodies

Knobe

Tedgård

et al. 2012

Eur J Pediatr

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Lupus anticoagulants in two children-bleeding due to nonphospholipid-dependent antiprothrombin antibodies.Knobe, Karin; Tedgård, Ulf; Ek, Torben; Sandström, Per-Erik; Hillarp, Andreas Link to publication Citation for published version (APA): Knobe, K., Tedgård, U., Ek, T., Sandström, P-E., & Hillarp, A. (2012). Lupus anticoagulants in two childrenbleeding due to nonphospholipid-dependent antiprothrombin antibodies. European Journal of Pediatrics, 171(9), 1383-1387. DOI: 10.1007/s00431-012-1737-1General rights Copyright and moral rights for the publications made accessible in the public portal are retained by the authors and/or other copyright owners and it is a condition of accessing publications that users recognise and abide by the legal requirements associated with these rights.• Users may download and print one copy of any publication from the public portal for the purpose of private study or research.• You may not further distribute the material or use it for any profit-making activity or commercial gain • You may freely distribute the URL identifying the publication in the public portal AbstractWe describe two children with significant bleeding; one with multiple ecchymoses and the other with scrotal bleeding. In both patients, the activated partial thromboplastin time (APTT) was prolonged with positivity for lupus anticoagulants (LA). However, the Owren prothrombin time (PT), usually insensitive for LA, was also prolonged. Presence of LA is associated with diverse clinical manifestations, most patients being asymptomatic while others present venous or arterial thrombosis. Bleeding in conjunction with lupus anticoagulants is rare and it is unusual to see prolongation of the Owren PT assay due to LA. An extended laboratory investigation of one of the patient's plasma revealed not only LA but also a specific non-phospholipid dependent anti-prothrombin antibody causing an acquired hypoprothrombinaemia. Conclusion: It is likely that the low prothrombin activity and not the LA caused the bleeding. The bleeding signs and symptoms in both patients subsided when the PT was normalised although the prolonged APTT and the LA remained.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Lupus anticoagulants in two children—bleeding due to nonphospholipid-dependent antiprothrombin antibodies

Knobe

Tedgård

et al. 2012

Eur J Pediatr

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show abstract

“…However, hemorrhage due to abnormalities in the coagulation system is a rare complication [1, 2]. Although lupus anticoagulant (LA), which is infrequently reported to be in association with MM, is commonly a risk factor for arterial or venous thrombosis, bleeding tendencies in patients with LA are strongly related to a low prothrombin activity [3–6]. Acquired hypoprothrombinemia with LA, also called LA hypoprothrombinemia syndrome (LAHPS), is a rare disease which appears mostly in young females with systemic lupus erythematosus or in healthy children after viral infection and is usually associated with the presence of antiprothrombin antibodies [7].…”

mentioning

confidence: 99%