Lupus anticoagulant-hypoprothrombinemia syndrome and similar diseases: experiences at a single center in Japan

• Published in: International journal of hematology Vol. 110; no. 2; pp. 197 - 204
• Main Authors: Ieko, Masahiro, Yoshida, Mika, Naito, Sumiyoshi, Ohmura, Kazumasa, Takahashi, Nobuhiko
• Format: Journal Article
• Language: English
• Published: Tokyo Springer Japan 01-08-2019; Springer Nature B.V
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Antibodies; Anticoagulants; Autoantibodies - blood; Autoantibodies - immunology; Bleeding; Child; Child, Preschool; Coagulation; Enzyme-Linked Immunosorbent Assay; Factor VIII - immunology; Female; Hematology; Hemophilia; Hemorrhage - epidemiology; Hemorrhage - etiology; Humans; Hypoprothrombinemias - epidemiology; Hypoprothrombinemias - etiology; Hypoprothrombinemias - immunology; Japan - epidemiology; Lupus; Lupus Coagulation Inhibitor - blood; Lupus Coagulation Inhibitor - immunology; Lupus Erythematosus, Systemic - blood; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - immunology; Male; Medicine; Medicine & Public Health; Middle Aged; Oncology; Original Article; Prothrombin; Prothrombin - analysis; Prothrombin - immunology; Risk analysis; Risk factors; Signs and symptoms; Syndrome; Systemic lupus erythematosus; Thrombophilia - epidemiology; Thrombophilia - etiology; Thrombophilia - immunology; Japan; Prothrombin activity; Anti-prothrombin antibodies; Lupus anticoagulant; Anti-factor VIII antibodies
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-019-02674-2

Patients with lupus anticoagulant (LA), a thrombotic risk factor, along with decreased prothrombin (FII) activity are classified as lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) and occasionally show bleeding symptoms, although this is not essential for diagnosis. We treated 20 cases of LAHPS over a 3-year period. Median FII activity was 20.9% and the anti-prothrombin antibody (anti-II Ab), shown by ELISA findings, was detected in 55%. Bleeding symptoms were observed in 20%, although that finding was not correlated with FII activity or anti-FII Ab quantity. We also observed 21 LA cases with decreased activity of coagulation factors other than FII, which we have designated LAHPS-like syndrome (LLS). Among LLS patients, anti-FII Ab and bleeding symptoms were seen in 47.6% and 14.3%, respectively. Our findings suggest that bleeding in LAHPS and LLS cannot be explained only by FII activity decreased by anti-FII Ab. Low FVIII activity and the anti-FVIII antibody (anti-FVIII Ab) were detected in some LAHPS and LLS patients, making it difficult to distinguish those from acquired hemophilia A cases. Detection of anti-FVIII Ab quantity by ELISA may be useful for accurate determination, as that was not performed in our LAHPS or LLS patients.