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Vol. 54. Núm. 5.
Páginas 444-449 (mayo 2001)
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Vol. 54. Núm. 5.
Páginas 444-449 (mayo 2001)
Acceso a texto completo
Anticuerpos antifosfolípido en población pediátrica asintomática
Antiphospholipid antibodies in asymptomatic pediatric patients
Visitas
9514
C. Aguilar Francoa,
Autor para correspondencia
caraguilar@excite.com

Correspondencia: Servicio de Hematología. Hospital General del INSALUD. P.° de Santa Bárbara, s/n. 42002 Soria
, J.F. Lucía Cuestab
a Servicio de Hematología. Hospital General del INSALUD. Soria
b Servicio de Hematología. Hospital Miguel Servet. Zaragoza
Este artículo ha recibido
Información del artículo
Antecedentes

El hallazgo de alargamientos del tiempo de tromboplastina parcial activado (TTPA) con criterios de anticoagulante lúpico (AL) es un hecho poco frecuente en niños asintomáticos que con frecuencia precede a ciertos tipos de cirugía y posee un comportamiento clínico benigno.

Pacientes y métodos

Se ha realizado un análisis de las características biológicas y clínicas de 13 niños con anticuerpos antifosfolípidos (APLA) (media de edad al diagnóstico 5 años) diagnosticados entre enero de 1996 y septiembre de 2000 a los que se realizó un seguimiento prospectivo (mediana, 16 meses; extremos, 15-60). Se realizaron determinaciones de AL por técnicas coagulométricas según los criterios de la International Society for Thrombosis and Haemostasis (ISTH) y anticuerpos anticardiolipina (ACA) y anti-β2-glucoproteína I por enzimoinmunoanálisis (ELISA).

Resultados

Todos los casos de anticoagulante lúpico estudiados se diagnosticaron tras investigación de un alargamiento del TTPA detectado con anterioridad a cirugía (adenoidectomía, 8 casos; orquidopexia, 1 caso; cirugía oftalmológica, 1 caso), asociado a alguna infección vírica (mononucleosis infecciosa, 1 caso) o como hallazgo casual en una analítica de rutina (2 casos). Todos ellos eran de tipo primario y un 53,6 % tuvieron carácter transitorio. Los ACA-IgG, anti-β2-glucoproteína I fueron negativos en todos los casos. El 30,7% presentaron valores ligeramente reducidos de factor XII:C (media, 38,2 U/dl). El diagnóstico de APLA no se vio acompañado de manifestaciones clínicas relacionadas con éstos ni tampoco se comunicó hemorragia posquirúrgica en ningún caso.

Conclusiones

Los APLA primarios representan un hallazgo poco frecuente en la población pediátrica asintomática que se ha descrito con relativa frecuencia en el preoperatorio de determinados tipos de cirugía (adenoidectomía y amigdalectomía) o infecciones víricas. Con frecuencia se trata de fenómenos transitorios, de muy escasa relevancia clínica y que pueden acompañarse de valores ligeramente reducidos de factor XII, por lo que debe establecerse el diagnóstico diferencial con el déficit leve de ese factor.

Palabras clave:
Anticoagulante lúpico
Anticuerpos antifosfolípidos
Niños
Background

Findings of prolonged activated partial thromboplastin time (APTT) and lupus anticoagulant are rare in asymptomatic children and are often preceded by certain types of surgery. Clinical behaviour is usually favorable.

Patients and methods

We assessed the biological and clinical features of antiphospholipid antibodies found in 13 children diagnosed between January 1996 and September 2000 (mean age at diagnosis: 5 years). The patients were prospectively followed- up for a median of 16 months (range: 15-60). The diagnosis of lupus anticoagulant was based on the guidelines of the International Society of Thrombosis and Hematosis and included coagulation-based assays as well as enzyme-linked immunosorbent assay for anticardiolipin and anti-beta 2 glycoprotein I.

Results

In all patients lupus anticoagulant was detected after investigation of prolonged APTT prior to surgery (adenoidectomy in eight patients, orchidopexy in one and eye surgery in one). The antibody was associated with infectious mononucleosis in one patient and was detected during routine laboratory investigations in two. All antibodies were primary and 53.6 % of events were transient. In all patients lupus anticoagulant IgG and anti-beta 2 glycoprotein I were negative. Slightly reduced factor XII:C plasma concentrations (mean: 38.2 UI/dl) were found in 30.7 % of the patients. No clinical manifestations of antiphospholipid symptoms were associated with the diagnosis of antiphospholipid antibodies and none of the patients experienced bleeding after surgery.

Conclusions

Primary antiphospholipid antibodies were infrequent in asymptomatic pediatric patients and were typically associated with certain types of surgery (adenoidectomy, tonsillectomy) or viral infections. They were usually transient and clinically irrelevant. These antibodies may be associated with slightly reduced plasma concentrations of factor XII and consequently a differential diagnosis with a mild factor XII deficiency should be considered.

Key words:
Lupus anticoagulant
Antiphospholipid antibodies
Children
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Biblografía
[1.]
G.R.V. Hughes.
Thrombosis, abortion, cerebral disease and the lupus anticoagulant.
Br Med J, 287 (1983), pp. 1088-1089
[2.]
H.P. McNeil, R.J. Simpson, C.N. Chesterman, S.A. Krilis.
Antiphospholipid antibodies are directed against a complex antigen that includes a lipid-binding inhibitor of coagulation: beta2 glycoprotein I (apolipoprotein H).
Proc Natl Acad Sci USA, 87 (1990), pp. 4120-4124
[3.]
E.M. Bevers, M. Galli, T. Barbui, P. Comfurius, R.F.A. Zwaal.
Lupus anticoagulant IgGs (LA) are not directed to phospholipids only, but to a complex of lipid-bound human prothrombin.
Thromb Haemostas, 66 (1991), pp. 629-632
[45.]
J.D. Oosting, R.H.W.M. Derksen, I.W.G. Bobbink, T.M. Hackeng, B.N. Bouma, K.I. De Groot.
Antiphospholipid antibodies directed against a combination of phospholipids with prothrombin against a combination of phospholipids with prothrombin, protein C or protein S: an explanation for their pathogenetic.
mechanism? Blood, 81 (1993), pp. 2618-2625
[5.]
S. Shibata, P.C. Harpel, A. Ghavari, J. Rand, H. Fillit.
Auto antibodies to heparin from patients with antiphospholipid antibody syndrome inhibit formation of antithrombin III-thrombin complexes.
Blood, 83 (1994), pp. 2532-2540
[6.]
P.G. De Groot, J.D. Oosting, R.H.W.M. Derksen.
Antiphospholipid antibodies: specificity and pathophysiology.
Baillieres Clin Haematol, 6 (1993), pp. 691-709
[7.]
K.S. Ginsberg, M.H. Liang, I. Newcomer, S.Z. Goldhaber, P.H. Schur, C.H. Hennekens.
Anticardiolipin antibodies and the risk for ischemic stroke and venous thromboembolism.
Ann Intern Med, 117 (1992), pp. 303-308
[8.]
M.H. Rosove, P.M.C. Brewer.
Antiphospholipid thromboses:clinical course after the first thrombotic event in 70 patients.
Ann Intern Med, 117 (1992), pp. 303-308
[9.]
G. Finazzi, V. Brancaccio, M. Moia.
Natural history and risk factors for thrombosis in 360 patients with antiphospholipid.
Am J Med, 100 (1996), pp. 530-536
[10.]
R.M. Silver, T.F. Porter, I. Van Leeuween, G. Jeng, J.R. Scott, D.W. Branch.
Anticardiolipin antibodies: clinical consequences of “low titers”.
Obst Gynecol, 87 (1996), pp. 530-536
[11.]
P. Vila, M.C. Hernández, M.F. López-Fernández, J. Batlle.
Prevalence, follow-up and clinical significance of the anticardiolipin antibodies in normal subjects.
Thromb Haemost, 72 (1994), pp. 209-213
[12.]
M.J. Manco-Johnson, R. Nuss.
Lupusanticoagulant in children with thrombosis.
Am J Hematol, 48 (1995), pp. 240-243
[13.]
A.K. Singh, K.P. Rao, J. Kizer, J. Lazarchick.
Lupus anticoagulants in children.
Ann Clin Lab Sci, 18 (1988), pp. 384-387
[14.]
J.T. Brandt, D.A. Tripplet, B. Alving, I. Scharrer.
Criteria for the diagnosis of lupus anticoagulants: An update.
Thromb Haemost, 74 (1995), pp. 1185-1190
[15.]
K.E. V, K. Gravem, F. Wisloff.
Preparation of plasma for the detection of lupus anticoagulants and antiphospholipid antibodies.
Thromb Res, 66 (1992), pp. 43-53
[16.]
P. Thiagaraja, V. Pengo, S. Shapiro.
The use of dilute Russell viper venom time for the diagnosis of lupus anticoagulants.
Blood, 68 (1986), pp. 869-874
[17.]
J. Arnout, E. Huybrechts, M. V, J. Vermylen.
A new lupus anticoagulant neutralization test based on platelet-derived vesicles.
Br J Haematol, 80 (1992), pp. 341-346
[18.]
Da. Triplett, L.K. V, G.A. Unger.
A hexagonal (II) phase phospholipid neutralization assay for lupus anticoagulant identification.
Thromb Haemost, 70 (1993), pp. 787-793
[19.]
S. Loizou, J.D. McCrea, A.C. Rudge, R. Reynolds, C. Boyle, E.N. Harris.
Measurement of anticardiolipin antibodies by an enzyme linked immunosorbent assay: standardization and quantitation of results.
Clinical and Experimental Immunology, 62 (1985), pp. 738-745
[20.]
M. Galli, G. Finazzi, F. Norbis, S. Marziali, R. Marchioli, T. Barbui.
The risk of thrombosis in patients with lupus anticoagulant is predicted by their specific coagulation profile.
Thromb Haemostas, 81 (1999), pp. 695-700
[21.]
R. Nuss, T. Hays, M.J. Manco-Johnson.
Childhood thrombosis.
Pediatrics, 96 (1995), pp. 291-294
[22.]
M.J. Manco-Johnson, R. Nuss.
Lupus anticoagulant in children.
Am J Hematol, 48 (1995), pp. 240-243
[23.]
C. Burk, L. Miller, S. Handler, A. Cohen.
Preoperative history and coagulation screening in children undergoing tonsillectomy.
Pediatrics, 89 (1992), pp. 691-695
[24.]
F.J. García-Callejo, L. Pardo Mateu, M.M. Velert Vila, M. Orts Alborch, R. Monzo Gandía, J. Marco Algarra.
Utilidad de las pruebas preoperatorias de coagulación en la prevención de la hemorragia post-amigdalectomía en niños.
Acta Otorrinolaringol Esp, 48 (1997), pp. 473-478
[25.]
M.J. Manco-Johnson.
Antiphospholid antibodies in children.
Semin Thromb Haemostas, 24 (1998), pp. 591-598
[26.]
J.C. Bernini, G.R. Buchanan, J. Ashcraft.
Hypoprothrombinaemia and severe hemorrhage associated with lupus anticoagulant.
J Pediatr, 123 (1993), pp. 937-939
[27.]
K.E. Hansen, J. Arnason, A.J. Bridges.
Autoantibodies and common viral illnesses.
Semin Arthritis Rheum, 27 (1998), pp. 263-271
[28.]
O. Vaarala.
Binding profiles of anticardiolipin antibodies in sera from patients with SLE and infectious diseases.
J Autoimmun, 4 (1991), pp. 819-830
[29.]
P. Nguyên, J. Reynaud, P. Pouzol, M. Munzer, O. Richard, P. Francois.
Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children.
Eur J Paediatr, 153 (1994), pp. 646-649
[30.]
M.J. Manco-Johnson, R. Nuss, N. Key, C. Moertel, L. Jacobson, S. Meech.
Lupus anticoagulant and protein S deficiency in children with postvaricella purpura fulminans or thrombosis.
J Pediatr, 128 (1996), pp. 319-323
[31.]
M.E. Martinuzzo, R.R. Forastiero, L.O. Carreras.
Anti-beta2 glycoprotein I antibodies: detection and association with thrombosis.
Br J Haematol, 89 (1995), pp. 397-402
[32.]
O. Amengual, T. Atsumi, M.A. Khamashta, T. Koike, G.R.V. Hughes.
Specificity of ELISA for antibodies to b2-glycoprotein I in patients with antiphospholipid syndrome.
Br J Rheumatol, 35 (1996), pp. 1239-1243
[33.]
A.R. Cabral, M.C. Amigo, J. Cabiedes, D. Alarcón-Segovia.
The antiphospholipid/cofactor syndrome: a primary variant with antibodies to b2-glycoprotein I but no antibodies detectable in standard antiphospholipid assay.
Am J Med, 101 (1996;), pp. 472-481
[34.]
M. Galli, G. Finazzi, E.M. Bevers, T. Barbui.
Kaolin clotting time and dilute Russell’s viper venom time distinguish between prothrombin-dependent and b2-glycoprotein I-dependent antiphospholipid antibodies.
Blood, 86 (1995), pp. 617-623
[35.]
L.P. Clyne, Y. Yen, N.S. Kriz, M.G. Breitenstein.
The lupus anticoagulant: high incidence of negative mixing studies in a human immunodeficiency virus-positive population.
Arch Pathol Lab Med, 117 (1993), pp. 595-601
[38.]
I. Jennings, S. Kitchen, T.A.L. Woods, F.E. Preston, M. Greaves.
(on behalf of the UK National External Quality Assessment Scheme for Blood Coagulation). Potentially clinically important inaccuracies in testing for the lupus anticoagulant: an analysis of results from three surveys of the UK National Assessment Scheme (NEQAS) for Blood Coagulation.
Thromb Haemostas, 77 (1997), pp. 934-937
[37.]
M.J. Gallimore, D.W. Jones, M. Winter.
Factor XII determinations Factor XII determinations antibodi.
Thromb Haemost, 79 (1998), pp. 87-90
[38.]
D.W. Jones, M.J. Gallimore, M. Winter.
Pseudo factor XII deficiency and phospholipid antibodies.
Thromb Haemost, 75 (1996), pp. 696-697
[39.]
D.W. Jones, M.J. Gallimore, S.L. Harris, M. Winter.
Antibodies to factor XII associated to lupus anticoagulant.
Thromb Haemost, 81 (1999), pp. 387-390
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