Rituximab in lupus anticoagulant hypoprothrombinemia syndrome: A case report

Abstract

Background

Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare autoimmune condition characterized by acquired prothrombin (FII) deficiency associated with antiphospholipid syndrome (APS) and life-threatening bleeding. We present the case of a 34-year-old woman with heavy menstrual bleeding (HMB), positive Lupus anticoagulant (LA) test, and high titer anticardiolipin antibodies Immunoglobulin G (ACA IgG) and anti-β2 glycoprotein I antibodies IgG (antiB2GPI IgG). Severe iron deficiency anemia necessitated recurrent blood transfusions and intravenous iron infusions from 2018 to 2021.

Results

In January 2022, she was admitted to our clinic. Von Willebrand disease screening and platelet function analysis (PFA100) were normal. FII and FIX deficiencies were detected, without factor IX inhibitors. Anti-phosphatidylserine/prothrombin antibodies were confirmed by Padua University lab. To reduce antibody titers and menstrual bleeding, immunosuppressive therapy (Rituximab 375 mg/m2 weekly ×4 weeks) and hormonal therapy (desogestrel 75 mcg/day) were initiated.

Conclusion

After 1-year, complete remission of clinical symptoms was achieved, with normalization of FII and FIX values and moderate reduction of aPS/PT titers, especially IgM isotype.

Keywords

Lupus anticoagulant LAHPS rituximab bleeding

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References

Rapaport

Ames

Duvall

. A plasma coagulation defect in systemic lupus erythematosus arising from hypoprothrombinemia combined with antiprothrombinase activity. Blood 1960; 15: 212–227.

Miyakis

Lockshin

Atsumi

, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemostasis 2006; 4: 295–306.

Devreese

KMJ

Ortel

Pengo

, et al. Laboratory criteria for antiphospholipid syndrome: communication from the SSC of the ISTH. J Thromb Haemostasis 2018; 16: 809.

Vandevelde

Chayoua

de Laat

, et al. Added value of antiphosphatidylserine/prothrombin antibodies in the workup of thrombotic antiphospholipid syndrome: communication from the ISTH SSC Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibodies. J Thromb Haemostasis 2022; 20: 2136–2150.

Bajaj

Rapaport

Fierer

, et al. A mechanism for the hypoprothrombinemia of the acquired hypoprothrombinemia-lupus anticoagulant syndrome. Blood 1983; 61(4): 684.

Bajaj

Rapaport

Barclay

, et al. Acquired hypoprothrombinemia due to non-neutralizing antibodies to prothrombin: mechanism and management. Blood 1985; 65(6): 1538.

Piliania

Suri

Jindal

, et al. Lupus anticoagulant hypoprothrombinemia syndrome associated with systemic lupus erythematosus in children: report of two cases and systematic review of the literature. Rheumatol Int 2018; 38(10): 1933–1940.

Mazodier

Arnaud

Mathian

, et al. Lupus anticoagulant-hypoprothrombinemia syndrome: report of 8 cases and review of the literature. Medicine (Baltim) 2012; 91(5): 251–260.

Cetin Gedik

Siddique

Aguiar

. Rituximab use in pediatric lupus anticoagulant hypoprothrombinemia syndrome – report of three cases and review of the literature. Lupus 2018; 27(7): 1190–1197.

10.

Chinnaraj

Planer

Pengo

, et al. Discovery and characterization of 2 novel subpopulations of aPS/PT antibodies in patients at high risk of thrombosis. Blood Adv 2019; 3(11): 1738–1749.