Journal Information
Vol. 4. Issue 2.
Pages 74-76 (March - April 2008)
Share
Share
Download PDF
More article options
Vol. 4. Issue 2.
Pages 74-76 (March - April 2008)
Clinical Cases
Full text access
Acquired Inhibitors of Coagulation in a Patient With Systemic Lupus Erythematosus and Antiphospholipid Antibodies: Response to Rituximab
Inhibidores adquiridos de la coagulación en una paciente con lupus eritematoso sistémico y anticuerpos antifosfolipídicos: respuesta a rituximab
Visits
3640
Enrique Ortiz Jiméneza,
Corresponding author
drortizjimenez@msn.com

Correspondence: Hospital General de Durango.Avda. 5 de Febrero esquina con Norman Fuentes s/n, zona centro. Durango. P 34000. México.
, Sergio Loera Fragosoa, Marina Rull Gabayetb
a Hospital General de Durango, Durango, Mexico
b Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Distrito Federal, Mexico
This item has received
Article information

Hematologic signs of systemic lupus erythematosus are diverse (SLE). Although a delayed coagulation time is anti-phospholipid antibody related, thrombotic events are the usual clinical manifestation. Spontaneous appearance of circulating anticoagulant in the absence of a previous coagulation disorder is secondary to the development of antibodies to factors II, V, VIII, IX, XI, XII, vonWillebrand, and other membrane glucoproteins, all of them uncommon causes (1 case per million persons per year) of life threatening coagulopathies. We report a case of SLE and antiphospholipid antibodies in a woman with a hemorrhagic syndrome, probably caused by multiple antibodies to coagulation factors, unresponsive to steroids and high-dose immunosupressive therapy and a favorable response to rituximab.

Key words:
Systemic lupus erythematosus
Acquired coagulation inhibitors
Lupus anticoagulants
Rituximab

Las manifestaciones hematológicas de los pacientes con lupus eritematoso sistémico (LES) son diversas. Aunque el desarrollo de anticuerpos antifosfolipídicos se asocia con una prolongación in vitro de los tiempos de coagulación, las manifestaciones clínicas suelen ser trombóticas. Los anticoagulantes circulantes que aparecen en pacientes sin anomalías previas de la coagulación son consecuencia del desarrollo de autoanticuerpos contra los factores II, V, VII, VIII y IX, XI, XIII, factor de Von Willebrand u otras glucoproteínas de membrana. Todos ellos condicionan coagulopatías poco comunes, 1 caso por millón de personas por año, y que pueden comprometer seriamente la vida de los pacientes. Presentamos el caso de una mujer con LES y anticuerpos antifosfolipídicos, que presentó síndrome de Evans, como primera manifestación, y posteriormente desarrolló un síndrome hemorrágico, presumiblemente por anticuerpos contra múltiples factores de la cascada de coagulación, sin respuesta a dosis altas de esteroides e inmunosupresores, que finalmente respondió a rituximab.

Palabras clave:
Lupus eritematoso sistémico
Inhibidores adquiridos de la coagulación
Anticoagulantes circulantes
Rituximab
Full text is only aviable in PDF
References
[1.]
A.J. Cohen, C.M. Kessler.
Acquired inhibitors.
Bailleres Clin Haematol, 9 (1996), pp. 331-354
[2.]
L.N. Boggio, D. Green.
Acquired hemophilia.
Rev Clin Exp Hematol, 5 (2001), pp. 389-404
[3.]
C.R.M. Hay, T.P. Baglin, P.W. Collins, F.G.H. Hill, D.M. Keeling.
The diagnosis and management of factor VIII and IX inhibitors: a guideline from the UK Hemophilia Center Doctors’ Organization.
Br J Haematol, 111 (2000), pp. 78-90
[4.]
A.E. Morrison.
Acquired hemophilia and its management.
Br J Haematol, 89 (1995), pp. 231-236
[5.]
R. Cervera, J.C. Piette, J. Font, M.A. Khamashta, Y. Shoenfeld, M.T. Camps, et al.
Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expressions in a cohort of 1000 patients.
Arthritis Rheum, 46 (2002), pp. 1019-1027
[6.]
G. Espinosa, R. Cervera, J. Font, Y. Shoenfeld.
Antiphospholipid syndrome: pathogenic mechanisms.
Autoimmun Rev, 2 (2003), pp. 86-93
[7.]
M.R. Clatworthy, D.R.W. Jayne.
Acquired hemophilia in association with ANCA-associated vasculitis: Response to rituximab.
Am J Kidney Dis, 47 (2006), pp. 680-682
[8.]
H.S. Lipkind, J.D. Kurtis, R. Powrie, M.W. Carpenter.
Acquired von Willebrand disease: Management of labor and delivery with intravenous dexamethasone, continuous factor concentrate and immunoglobulin infusion.
Am J Obstet Gynecol, 192 (2005), pp. 2067-2070
[9.]
T. Ishikawa, N. Tsukamoto, M. Suto, H. Uchiumi, H. Mitsuhashi, A. Yokohama, et al.
Acquired hemophilia in a patient with systemic lupus erythematosus.
Internal Medicine, 40 (2001), pp. 541-543
[10.]
A. Abdallah, D. Coghlan, E. Duncan, S.D. Chunilal, J.V. Lloyd.
Rituximabinduced long-term remission in patients with refractory acquired hemophilia.
J Thromb Haemost, 3 (2005), pp. 2589-2590
[11.]
J.H. Anilik, M. Aringer.
New treatments for SLE: cell-depleting and anticytokine therapies.
Best Pract Res Clin Rheumatol, 19 (2005), pp. 859-878
Copyright © 2008. Sociedad Española de Reumatología and Colegio Mexicano de Reumatología
Idiomas
Reumatología Clínica (English Edition)
Article options
Tools
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?