A Plasma Coagulation Defect in Systemic Lupus Erythematosus Arising from Hypoprothrombinemia Combined with Antiprothrombinase Activity

Abstract

A patient has been described with systemic lupus erythematosus and severe bleeding. Her bleeding was associated with a complex plasma coagulation disturbance consisting of profound hypoprothrombinemia plus an anticoagulant active against formed blood and tissue prothrombinase. The problem of the recognition of hypoprothrombinemia in the presence of this type of anticoagulant has been discussed in detail. An analysis of previously reported cases reveals that our patient’s findings are not unique. It appears that the plasma coagulation disturbances of systemic lupus erythematosus characteristically result from a mixture of anticoagulant activity and true hypoprothrombinemia. In an individual patient one or the other may predominate.

Diagnosis of lupus anticoagulant in the lupus anticoagulant‐hypoprothrombinemia syndrome: Report of two cases and review of the literature

“…Thus, our data and those described previously suggest that certain LA antibodies are specifically inhibited by hexagonal (II) phase phospholipids, or more specifically, by the complex prothrombin-hexagonal (II) phase phospholipids. The proposed mechanism of hypoprothrombinemia in LA-HPS considers that non-neutralizing antibodies bind prothrombin, resulting in the rapid clearance of prothrombin-anti-prothrombin antibody complexes from circulation [9]. Herein, we demonstrated the presence of anti-prothrombin antibodies in both patients.…”

“…On some occasions, it can present with hemorrhagic diathesis as a consequence of LA-HPS. Since its first descrip-tion in 1960 by Rapaport et al [9] in an 11-year-old girl with SLE and severe bleeding, the LA-HPS has been documented in the literature in 29 pediatric patients [1-3,10-23] and less frequently in adult patients [4,[24][25][26][27][28][29][30][31][32][33][34][35].…”

Lupus anticoagulant acquired hypoprothrombinemia syndrome in childhood: two distinct patterns and review of the literature

“…A total of 34 articles including case reports, case series and letters to editors between 1960 and 2014 were reviewed ( Table 2) [1][2][3][4][5][6][7][8][9][10][11][12][13]15,[17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36]. Fifteen case reports [6,8,13,[17][18][19][20][21][22][26][27][28][29][30]36] and 5 case series [1,...…”

“…However, bleeding diathesis associated with LA can be seen in children with acquired factor II (FII) (prothrombin) deficiency. Combined together, the condition is referred to as 'lupus anticoagulant hypoprothrombinemia syndrome' or LAHS [4][5][6][7][8][9][10][11][12][13]. Although intracranial bleeds in patients with LAHS secondary to autoimmune disorders (LAHS-AI) have been reported in adult patients [14,15], there have been no reports of severe intracranial hemorrhages in children.…”

Resolution of Hypoprothrombinemia‐Lupus Anticoagulant Syndrome (HLAS) after multidrug therapy with rituximab: a case report and review of the literature

“…In cases where the antibody persists, therapy has mainly consisted of immunosuppressive drugs. Corticosteroids remain the backbone of initial therapy [3,8,9,[12][13][14][15][17][18][19][20][21][22][23][24][25][26][27]. It is postulated that steroids decrease the clearance of prothrombin-antiprothrombin complexes [9].…”