POS1307 HEMATOLOGICAL MANIFESTATIONS IN THE PRESENCE OF ANTIPHOSPHOLIPID ANTIBODIES IN A PEDIATRIC COHORT

Abstract

BackgroundAntiphospholipid syndrome (APS) is an autoimmune disease characterized by thrombotic events (TEs) and/or pregnancy morbidity, in association with two consecutive positive determinations (at least 12 weeks apart) of antiphospholipid antibodies (aPLs). Several manifestations which are not considered clinical criteria of APS have been identified, including hematologic disorders. 1ObjectivesTo identify the different variables associated with the development of hematological manifestations in the presence of aPLs in a pediatric cohort.MethodsMulticentric historical cohort of children ≤ 18 years from three Spanish and one Italian tertiary referral hospitals with at least 2 positive determinations (≥12 weeks apart) of IgG/IgM anticardiolipin (aCL), IgG/IgM anti β2-glycoprotein I (aβ2GP) and/or LA. Demographic, clinical and analytical data were collected. A bivariate and multivariate analysis were carried out. Multicollinearity was also explored to build the model.Results131 children were included. Demographic, clinical, analytical data; and results of bivariant analysis are shown in Table 1.Table 1.Demographic characteristics; non-thrombotic clinical manifestations; immunologic laboratory findings; and bivariate analysis according to each specific entity and the occurrence of “hematological” manifestations. Categorical variables expressed as number (n) and percentage (%); Numerical variables with normal distribution expressed as mean and SD (standard deviation). * p<0.05.VariablesTotaln= 131Hematological manifestationsn= 55Absence of hematological manifestationsn= 76P-valueSex, female, n (%)85 (64.9)39 (70.9)46 (60.5)0.219Age, years, mean (SD)10.9 (5.1)10.8 (5.03)10.9 (5.2)0.974Rage, n (%)0.126 Caucasian105 (81.4)43 (79.6)62 (82.7) Maghreb9 (7)2 (3.7)7 (9.3) Latinoamerican8 (6.2)6 (11.1)2 (2.7) Asian5 (3.9)3 (5.6)2 (2.7)Family history of AIDs, n (%)33 (25.2)17 (33.3)16 (21.1)0.122SLE diagnosis, n (%)34 (26)20 (36.4)14 (18.4)0.021*Thrombotic events, n (%)16 (12.2)7 (12.7)9 (11.8)0.879Hematological, n (%)55 (42)--- Thrombocytopenia30 (22.9) Leucopenia16 (8.4) Hemolytic anemia7 (5.3) Evans syndrome2 (1.2)Neurological, n (%)26 (20)10 (18.2)16 (21.3)0.657 Chorea8 (6.1) Epilepsy5 (3.8) Migraine4 (3)Cutaneous, n (%)23 (17.6)6 (10.9)17 (22.4)0.089 Raynaud17 (13) Livedo5 (3.1)Cardiac, n (%)22 (16.8)12 (21.8)10 (13.2)0.191Renal, n (%)2 (1.5)0 (0)2 (2.6)0.225aCL, n (%) IgG54 (41.2)24 (43.6)30 (39.5)0.633 IgM31 (23.7)20 (36.4)11 (14.5)0.004*aB2GP, n (%) IgG59 (45)29 (52.7)30 (39.5)0.132 IgM24 (18.3)16 (29.1)8 (10.5)0.007*LA, n (%)76 (58.5)37 (67.3)39 (52)0.081aPL profile, n (%) Simple76 (58)25 (45.5)51 (67.1)0.008* Double30 (22.9)13 (23.6)17 (22.4) Triple25 (19.1)17 (30.9)8 (10.5)ANA +, n (%)72 (59.5)33 (66)39 (54.9)0.222The multivariate analysis identified as independent risk factors to develop a hematological manifestation, children with: SLE diagnosis [OR 1.2, 95 CI (0.5-5.8), p= 0.015], cutaneous manifestations [OR 1.01, 95 CI (0.6 -2.8), p= 0.091], LA + [OR 0.8, 95 CI (0.4 -3.6), p= 0.058], and IgG aβ2GP + [OR 0.9, 95 CI (0.4 - 3.9), p= 0.048]. Sex, age, familiar history of AIDs or IgM B2GP+ did not show a higher risk.ConclusionNon-criteria manifestations, specially hematologic disorders, are the most frequent events in the presence of aPLs in our cohort. Cutaneous manifestations, a positive personal history of SLE, LA positivity and IgG aβ2GP positivity were associated with a higher risk of developing hematological manifestations. Therefore, their inclusion as APS classification criteria should be considered.