Report of a factor VIII inhibitor in a patient with autoimmune lymphoproliferative syndrome

被引：0
作者：
Fang, BS
Sneller, MC
Straus, SE
Frenkel, L
Dale, JK
Rick, ME
机构：
[1] NIH, Warren Grant Magnuson Clin Ctr, Hematol Serv, Bethesda, MD 20892 USA
[2] NIAID, Lab Immunoregulat, NIH, Bethesda, MD 20892 USA
[3] NIAID, Lab Clin Invest, NIH, Bethesda, MD 20892 USA
[4] Scott & White Mem Hosp & Clin, San Antonio, TX USA
来源：
AMERICAN JOURNAL OF HEMATOLOGY | 2000年 / 64卷 / 03期
关键词：
autoimmune lymphoproliferative syndrome; factor VIII inhibitor; bleeding disorder;
D O I：
10.1002/1096-8652(200007)64:3<214::AID-AJH14>3.3.CO;2-K
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
The occurrence of factor VIII inhibitors in non-hemophilic patients is a rare event with a potentially lethal outcome. Despite its infrequent occurrence, the association of this inhibitor with multiple autoimmune diseases is well recognized. We report the case of a patient with the recently described autoimmune lymphoproliferative syndrome (ALPS) who developed an inhibitor to factor VIII, ALPS is a disease characterized by defective lymphocyte apoptosis due to inherited mutations in genes that regulate apoptosis, with the resulting enlargement of lymphoid organs and a variety of autoimmune manifestations. Published 2000 Wiley-Liss, Inc.(1)
引用
收藏
页码：214 / 217
页数：4
相关论文
共 20 条
  • [1] Correction of Fas (CD95) deficiency by haploidentical bone marrow transplantation
    Benkerrou, M
    LeDeist, F
    deVillartay, JP
    CaillatZucman, S
    RieuxLaucat, F
    Jabado, N
    CavazzanaCalvo, M
    Fischer, A
    [J]. EUROPEAN JOURNAL OF IMMUNOLOGY, 1997, 27 (08) : 2043 - 2047
  • [2] Missense mutations in the Fas gene resulting in autoimmune lymphoproliferative syndrome: A molecular and immunological analysis
    Bettinardi, A
    Brugnoni, D
    QuirosRoldan, E
    Malagoli, A
    LaGrutta, S
    Correra, A
    Notarangelo, LD
    [J]. BLOOD, 1997, 89 (03) : 902 - 909
  • [3] Deficiency of the Fas apoptosis pathway without Fas gene mutations in pediatric patients with autoimmunity/lymphoproliferation
    Dianzani, U
    Bragardo, M
    DiFranco, D
    Alliaudi, C
    Scagni, P
    Buonfiglio, D
    Redoglia, V
    Bonissoni, S
    Correra, A
    Dianzani, I
    Ramenghi, U
    [J]. BLOOD, 1997, 89 (08) : 2871 - 2879
  • [4] Fas gene mutations in the Canale-Smith syndrome, an inherited lymphoproliferative disorder associated with autoimmunity
    Drappa, J
    Vaishnaw, AK
    Sullivan, KE
    Chu, JL
    Elkon, KB
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1996, 335 (22) : 1643 - 1649
  • [5] GREEN D, 1993, THROMB HAEMOSTASIS, V70, P753
  • [6] GREEN D, 1981, THROMB HAEMOSTASIS, V45, P200
  • [7] KASPER CK, 1975, THROMB DIATH HAEMOST, V34, P869
  • [8] Clinical, immunological, and pathological consequences of Fas-deficient conditions
    LeDeist, F
    Emile, JF
    RieuxLaucat, F
    Benkerrou, M
    Roberts, I
    Brousse, N
    Fischer, A
    [J]. LANCET, 1996, 348 (9029) : 719 - 723
  • [9] Fas and the art of lymphocyte maintenance
    Lenardo, MJ
    [J]. JOURNAL OF EXPERIMENTAL MEDICINE, 1996, 183 (03) : 721 - 724
  • [10] COMBINATION IMMUNOSUPPRESSIVE THERAPY AFTER FACTOR-VIII INFUSION FOR ACQUIRED FACTOR-VIII INHIBITOR
    LIAN, ECY
    LARCADA, AF
    CHIU, AYZ
    [J]. ANNALS OF INTERNAL MEDICINE, 1989, 110 (10) : 774 - 778
12