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
  • [11] Pathological findings in human autoimmune lymphoproliferative syndrome
    Lim, MS
    Straus, SE
    Dale, JK
    Fleisher, TA
    Stetler-Stevenson, M
    Strober, W
    Sneller, MC
    Puck, JM
    Lenardo, MJ
    Elenitoba-Johnson, KSJ
    Lin, AY
    Raffeld, M
    Jaffe, ES
    [J]. AMERICAN JOURNAL OF PATHOLOGY, 1998, 153 (05) : 1541 - 1550
  • [12] ACQUIRED HEMOPHILIA - A NATURAL-HISTORY STUDY OF 16 PATIENTS WITH FACTOR-VIII INHIBITORS RECEIVING LITTLE OR NO THERAPY
    LOTTENBERG, R
    KENTRO, TB
    KITCHENS, CS
    [J]. ARCHIVES OF INTERNAL MEDICINE, 1987, 147 (06) : 1077 - 1081
  • [13] Schulman S, 1996, THROMB HAEMOSTASIS, V76, P344
  • [14] A PROSPECTIVE-STUDY OF TREATMENT OF ACQUIRED (AUTOIMMUNE) FACTOR-VIII INHIBITORS WITH HIGH-DOSE INTRAVENOUS GAMMA-GLOBULIN
    SCHWARTZ, RS
    GABRIEL, DA
    ALEDORT, LM
    GREEN, D
    KESSLER, CM
    [J]. BLOOD, 1995, 86 (02) : 797 - 804
  • [15] THE FAS ANTIGEN IS INVOLVED IN PERIPHERAL BUT NOT THYMIC DELETION OF T-LYMPHOCYTES IN T-CELL RECEPTOR TRANSGENIC MICE
    SINGER, GG
    ABBAS, AK
    [J]. IMMUNITY, 1994, 1 (05) : 365 - 371
  • [16] A NOVEL LYMPHOPROLIFERATIVE AUTOIMMUNE SYNDROME RESEMBLING MURINE LPR/GLD DISEASE
    SNELLER, MC
    STRAUS, SE
    JAFFE, ES
    JAFFE, JS
    FLEISHER, TA
    STETLERSTEVENSON, M
    STROBER, W
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1992, 90 (02) : 334 - 341
  • [17] Clinical, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis
    Sneller, MC
    Wang, J
    Dale, JK
    Strober, W
    Middelton, LA
    Choi, YN
    Fleisher, TA
    Lim, MS
    Jaffe, ES
    Puck, JM
    Lenardo, MJ
    Straus, SE
    [J]. BLOOD, 1997, 89 (04) : 1341 - 1348
  • [18] An inherited disorder of lymphocyte apoptosis: The autoimmune lymphoproliferative syndrome
    Straus, SE
    Sneller, M
    Lenardo, MJ
    Puck, JM
    Strober, W
    [J]. ANNALS OF INTERNAL MEDICINE, 1999, 130 (07) : 591 - 601
  • [19] Inherited human Caspase 10 mutations underlie defective lymphocyte and dendritic cell apoptosis in autoimmune lymphoproliferative syndrome type II
    Wang, J
    Zheng, LX
    Lobito, A
    Chan, FKM
    Dale, J
    Sneller, M
    Yao, X
    Puck, JM
    Straus, SE
    Lenardo, MJ
    [J]. CELL, 1999, 98 (01) : 47 - 58
  • [20] Fas ligand mutation in a patient with systemic lupus erythematosus and lymphoproliferative disease
    Wu, JG
    Wilson, J
    He, J
    Xiang, LB
    Schur, PH
    Mountz, JD
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1996, 98 (05) : 1107 - 1113
12