Disseminated cerebral thrombotic microangiopathy in a patient with adult's Still disease

被引:13
作者
Domingues, RB
Gama, AMC
Caser, EB
Musso, C
Santos, MCS
机构
[1] Escola Med Santa Casa Misericordia Vitoria, Vitoria, ES, Brazil
[2] UFES, Dept Clin Med, Vitoria, ES, Brazil
关键词
adult's Still disease; cerebral thrombotic microangiopathy; thrombotic thrombocitopenic purpura;
D O I
10.1590/S0004-282X2003000200018
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Objective: The aim of this report is to describe a fatal disseminated thrombotic micoangiopathy with renal, pancreatic, and cerebral involvement in a patient with recently diagnosed adult's Still disease (ASD). Case report: A previously healthy 15 year old girl was admitted to our hospital. According to the clinical and laboratory data the diagnosis of adult's still Disease was established. The treatment was begun and few days after an initial improvement a sudden neurologic deterioration with coma and seizures has occurred. Hours later the patient died. Clinical, laboratorial, and pathologic data will be presented. Conclusion: To our knowledge this is the second description of a fatal disseminated,cerebral thrombotic microangiopathy in a patient with adult's Still disease, but with a much more fulminating evolution than previously reported. Some etiopathogenic mechanisms could be shared in these two disorders explaining this coexistence.
引用
收藏
页码:259 / 261
页数:3
相关论文
共 11 条
[1]  
Boki KA, 1996, J RHEUMATOL, V23, P385
[2]  
Cines DB, 1998, BLOOD, V91, P3527
[3]   Enhanced endothelial cell apoptosis in splenic tissues of patients with thrombotic thrombocytopenic purpura [J].
Dang, CT ;
Magid, MS ;
Weksler, B ;
Chadburn, A ;
Laurence, J .
BLOOD, 1999, 93 (04) :1264-1270
[4]  
HARDIN RI, 1998, HARRISONS PRINCIPLES, P730
[5]  
MASSON C, 1986, REV RHUM, V53, P389
[6]   Thrombotic thrombocytopenic purpura and sporadic hemolytic-uremic syndrome plasmas induce apoptosis in restricted lineages of human microvascular endothelial cells [J].
Mitra, D ;
Jaffe, EA ;
Weksler, B ;
Hajjar, KA ;
Soderland, C ;
Laurence, J .
BLOOD, 1997, 89 (04) :1224-1234
[7]   ADULT STILLS DISEASE - MANIFESTATIONS, DISEASE COURSE, AND OUTCOME IN 62 PATIENTS [J].
POUCHOT, J ;
SAMPALIS, JS ;
BEAUDET, F ;
CARETTE, S ;
DECARY, F ;
SALUSINSKYSTERNBACH, M ;
HILL, RO ;
GUTKOWSKI, A ;
HARTH, M ;
MYHAL, D ;
SENECAL, JL ;
YEADON, C ;
ESDAILE, JM .
MEDICINE, 1991, 70 (02) :118-136
[8]   ADULT ONSET STILLS DISEASE - EXPERIENCE IN 23 PATIENTS AND LITERATURE-REVIEW WITH EMPHASIS ON ORGAN FAILURE [J].
REGINATO, AJ ;
SCHUMACHER, HR ;
BAKER, DG ;
OCONNOR, CR ;
FERREIROS, J .
SEMINARS IN ARTHRITIS AND RHEUMATISM, 1987, 17 (01) :39-57
[9]   ASPIRIN HEPATOTOXICITY AND DISSEMINATED INTRAVASCULAR COAGULATION [J].
SBARBARO, JA ;
BENNETT, RM .
ANNALS OF INTERNAL MEDICINE, 1977, 86 (02) :183-185
[10]   CONSUMPTION COAGULOPATHY ASSOCIATED WITH SYSTEMIC JUVENILE RHEUMATOID-ARTHRITIS [J].
SILVERMAN, ED ;
MILLER, JJ ;
BERNSTEIN, B ;
SHAFAI, T .
JOURNAL OF PEDIATRICS, 1983, 103 (06) :872-876