APPENDIX 1 Eight New Cases of LAHS and Review of the Literature: Clinical and Laboratory Data

Transcription

1 Supplementary Digital Content 1 Mazodier et al. Lupus Anticoagulant- Hypoprothrombinemia Syndrome: Report of 8 Cases and Review of the Literature Medicine (Baltimore). 2012;91(5). APPEDIX 1 Eight ew Cases of LAHS and Review of the Literature: Clinical and Laboratory Data First Author (year)/ref Sex/Age (yr) Bleeding Events Thrombotic and Obstetric Events Associated Disease aptt PT (sec) FII Level Anti- Factor II Ab apl Other Case 1/PR M/65 Intracerebral Deep venous thrombosis Primary APS (1.9 ) 11% acl IgG: 366 a-β2gp1 IgG: 12 (117 g/l) Case 2/PR F/13 Case 3/PR F/56 Gum, Post-dental extraction no SLE >4 18% 4% no Lupus-like syndrome (arthritis and skin lesion) (D) acl IgG: 9 a-β2gp1: D >4 (IR 1.98) 38% acl: Case 4/PR M/43 Intramuscular (t-trauma) no % 20% acl IgG: 10 a-β2gp1: D Case 5/PR M/48 o Case 6/PR M/57 Case 7/PR F/50 Hematoma (tsurgery) Metrorrhagia and (tsurgery) Arterial stenosis and thrombosis 3 miscarriages (medical history) Primary APS > (1.3 ) 35% D no 4 46% 12% SLE, obstetric APS (1.4 ) 33% Case 8/PR F/13 Metrorrhagia SLE % 20% Rapaport (1960)/[42] F/11 gum +++ IgG : 2 IgG : 23 acl IgG: +++ a-β2gp1 IgG: +++ acl IgG: 37 a-β2gp1: IgG+++ acl IgG: 8 acl IgM: 1.5 a-β2gp1 IgG: 2 acl IgG: 18 a-β2gp1: 66 no SLE 2 21% 10% D D 1

2 Perkins (1960)/[39] Biggs (1964)/[10] Gonyea (1968)/[21] Corrigan (1970)/[13] Bajaj (1983)/[6] Shaulian (1981)/[46] Follea (1981)/[19] Houbouyan (1984)/[28] Schwartz (1984)/[44] Harrison (1987)/[23] Simel (1987)/[47] Small (1988)/[48] Stormorken (1988)/[49] F/33 F/11 M/45 F/4 F/56 F/37 Menorrhagia, gum, Bleeding after tooth extraction, Hematuria, Severe Ecchymosis, major Postoperative Postoperative (hysterectomy) no Lupus-like syndrome D D 40% D D no Lupus-like syndrome D % D D Lupus-like syndrome D 2 10% D D no SLE D 43 (3 ) 30% D D Yes (D) SLE (2 ) < 1% D D no Viral 3 35 (2.5 ) 1% D no SLE + viral (1.7 ) 6% D Arterial and venous thrombosis, pulmonary embolism SLE 2 20% D D D no SLE 4 12% D D D M/39 o symptom no Lymphoma 3 D 20% D D F/2 M/14 M/42 Ecchymosis,, gum, Bleeding after tooth extraction gum no Viral (1.7 ) 3% D D no SLE (1.2 ) 23% D acl: no Drug 2 20 (1.6 ) 8% M/66 o symptom no no (1.1 ) 32% D M/22 F/40 gum Metrorrhagia,, acl IgG: acl IgM: acl IgG: VDRL+/TPHA- no SLE 3 23 (1.9 ) 4% D acl: 3 miscarriages at 12 SA (medical history) SLE 3 *20% 5% D VDRL+/TPHA- (15 g/l) (86 g/l) (114 g/l) 2

3 Baudo (1990)/[7] Hift (1991)/[24] Jaeger (1993)/[31] Bernini (1993)/[9] Humphries (1994)/[30] Peacock (1994)/[37] Eberhard (1994)/[17] Lee (1996)/[33] Williams (1996)/[53] Côté (1997)/[14] M/23 M/24 intramuscular no SLE 2.4 D 1% no SLE 3 16 (1.6 ) 2% D D M/3 o symptom no Viral 2 34% 16% F/4 o symptom no Viral 1.7 <5% 10% F/7 o symptom no Viral 3 45% 22% F/5 o symptom no Viral 3 6% 11% F/3 F/5 M/38 F/15 F/10 F/11 Ecchymosis,, gum, gastrointestinal gastrointestinal, subdural Menorrhagia, Hematuria, bruising, petechiae Petechiae, conjunctival hemorrhage acl: no Viral 3 >100 (>3 ) <1% D acl: 2 no Viral (1.3 ) 15% D acl: Myocardial infarction Primary APS 3 23 (1.8 ) 13% acl: 2 no SLE (1.8 ) 5% D VDRL+/TPHA- Deep venous thombosis SLE, pneumococcal infection (1.9 ) 12% D D no SLE (1.6 ) 4% D D F/2 Bruising Viral 2 23 (1.8 ) 14% acl: M/64 tbiopsy no Discoid lupus (1.2 ) 22% acl IgG: acl IgM: F/9 Epistaxis no Celiac disease 5 43 (3.2 ) 2% acl: (132 g/l) (115 g/l) 3

4 Becton (1997)/[8] Pernod (1997)/[40] Grau (1997)/[22] Vivaldi (1997)/[52] Moll (1997)/[36] Hudson (1997)/[29] Amiral (1997)/[1] de Larranaga (1999)/[16] Male (1999)/[35] Erkan (1999)/[18] F/8 M/2 bruising no no (1.3 ) 17% D acl: no Viral (1.8 ) 12% D acl: M/10 Elbow no Viral (1.2 ) 12% D acl: M/4 Hematemesis, no Viral (2.1 ) 7% D acl: M/40 Hematuria no no (1.2 ) F/17 M/43 Postcutaneous biopsy hemorrhage,, bruising, metrorrhagia Subdural 0.1 UI/mL no SLE (1.7 ) 30% D no SLE % D acl: a-β2gp1: acl IgG: 3 acl IgM: 1.2 acl IgG: acl IgM: no o (1.4 ) 19% D acl IgG: F/15 Menorrhagia Amaurosis fugax Primary APS (1.1 ) 0.27 U/mL F/6 Purpura no Adenovirus (1.2 ) 26% M/28 melena no Pneumonia, autoimmune hepatitis 3.3 D (1.6 ) 30% D : 34 acl IgG: 3 acl IgM: 1.5 acl: a-β2gp1: acl IgG: 22 a-β2gp1 IgG: 25 a-β2gp1 IgM: 2 F/4 Purpura no Adenovirus D D 10% D F/4 Bruising no Adenovirus M/4 Melena no Adenovirus M/8 Bruising no Adenovirus M/2 Hemorrhagic diarrhea no Adenovirus D. D D D D 9% D D 18% D D 5% D D 16% D F/19 Menorrhagia no SLE (1.3 ) 11% D F/47 Macroscopic acl IgG: acl IgM: no SLE (1.7 ) 21% D acl IgG: (102 g/l) (119 g/l) (32 g/l) (125 g/l) (117 g/l) 4

5 Holm (1999)/[27] Ayoub (1999)/[4] Schmugge (2001)/[43] Yacobovitch (2001)/[54] Baca (2002)/[5] Anderson (2003)/[2] Sellami (2004)/[45] Vinet (2006)/[51] Taddio (2007)/[50] Raflores (2007)/[41] F/76 F/26 F/4 F/13 Hematuria, menorrhagia, rectorrhagia Hematoma,,, gum Gum,,, intramuscular hemorrhage macroscopic. menorrhagia no Lymphoma (1 ) Miscarriage, preeclampsy (medical history) 0.12 U/mL SLE (4 ) 0.2 U/L D acl: no Viral 2 27% <10% D acl: a-β2gp1: no SLE 2 IR 2.5 5% D acl: no Lupus-like syndrome (2.3 ) 1% M/11 Epistaxis no SLE (2.2 ) 1% F/3 M/23 F/50 M/13 M/38 gum Subdural Macroscopic, menorrhagia,, purpura LAHS diagnosed 15 yr earlier,, : 10 no viral (1 ) 40% D Splenic infarction, pulmonary embolism Stroke Catheter-induced venous thrombosis SLE % 2% D SLE (20 yr earlier) SLE (3 yr earlier) SLE 3 IR IU/mL D acl IgG: acl IgM: a-β2gp1: D acl IgG : 2 acl IgM: a-β2gp1: 1.5 acl: a-β2gp1: acl IgG: acl IgM: D (1.3 ) 12% D D (1.23 ) Primary APS (1.3 ) 32% D acl: 0.20 IU/mL : 4 acl IgG: 4 (68 g/l) (122 g/l) (92 g/l) (93 g/l) 5

6 Clauser (2007)/[12] Davey (2007)/[15] Chung (2008)/[11] M/68 after minor trauma no Drug (quinidine) (1.2 ) 31% M/2 Bruising Viral (2.9) 2 IU/dL (: ) : 3 D D acl IgG: 1.3 M/34 no no no (1.6 ) 29% D D Appert-Flory (2010)/[3] F/2 bruising no Viral (6 ) <10% acl IgG: Abbreviations: a-β2gp1 = anti-β 2-glycoprotein1 antibodies, acl = anticardiolipin antibodies, APS = antiphospholipid syndrome, aptt = activated partial thromboplastin time, IR = international normalized ratio, = n-times the upper limit of the normal range, D = not documented, = ative, =, PR = present report, PT = prothrombin time, SLE = systemic lupus erythematosus, VDRL+/TPHA- = false-itive syphilis serology. 6