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Titolo:
Thrombotic thrombocytopenia purpura in a patient with systemic sclerosis
Autore:
Yusin, J; Lewin, K; Clements, P;
Indirizzi:
VA Med Ctr Phoenix, Dept Med, Sect Allergy & Immunol 3, Phoenix, AZ 85012 USA VA Med Ctr Phoenix Phoenix AZ USA 85012 Immunol 3, Phoenix, AZ 85012 USA Univ Calif Los Angeles, Div Pathol, Los Angeles, CA USA Univ Calif Los Angeles Los Angeles CA USA iv Pathol, Los Angeles, CA USA Univ Calif Los Angeles, Div Rheumatol, Los Angeles, CA USA Univ Calif Los Angeles Los Angeles CA USA Rheumatol, Los Angeles, CA USA
Titolo Testata:
JCR-JOURNAL OF CLINICAL RHEUMATOLOGY
fascicolo: 2, volume: 7, anno: 2001,
pagine: 106 - 111
SICI:
1076-1608(200104)7:2<106:TTPIAP>2.0.ZU;2-K
Fonte:
ISI
Lingua:
ENG
Soggetto:
HEMOLYTIC-UREMIC SYNDROME; SCLERODERMA RENAL CRISIS; VASCULITIS;
Keywords:
thrombotic thrombocytopenic purpura; thrombocytopenia; scleroderma; plasma exchange;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
15
Recensione:
Indirizzi per estratti:
Indirizzo: Yusin, J VA Med Ctr Phoenix, Dept Med, Sect Allergy & Immunol 3, 650 E Indian Sch Rd, Phoenix, AZ 85012 USA VA Med Ctr Phoenix 650 E Indian Sch Rd Phoenix AZ USA 85012 2 USA
Citazione:
J. Yusin et al., "Thrombotic thrombocytopenia purpura in a patient with systemic sclerosis", JCR-J CLIN, 7(2), 2001, pp. 106-111

Abstract

Thrombotic thrombocytopenic purpura (TTP) has been associated with scleroderma renal crises (SRC) in the past. However such reports markedly diminished after the onset of ACE inhibitor use. Recently, reports again have surfaced that describe scleroderma patients presenting with clinical evidence ofTTP We describe a 50-year-old female with longstanding limited cutaneous scleroderma who presented with hematochezia and thrombocytopenia along with other findings suggesting TTP. A colon biopsy revealed thrombi within the lumen. Her course was complicated by renal failure and hypertension that didnot respond to ACE inhibitor therapy alone. She improved after a course ofplasma exchange. She was discharged home only to return 2 months later with grand mal seizures and hypertension. During her course she developed adult respiratory distress syndrome. She again responded to plasma exchange andshe was discharged home. She has remained stable for 2 years. This report emphasizes the importance of fully evaluating patients with longstanding limited cutaneous scleroderma who present with renal failure, hypertension, and thrombocytopenia in association with multiorgan complications. All possible etiologies, including SRC, TTP, vasculitis, and sepsis should be considered. Tissue biopsies (in this case, a colon biopsy revealed thrombi within the vessel lumen) may prove beneficial in assisting with the diagnosis. For such patients who fail treatment with ACE inhibitors, plasmaexchange may be considered.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 17/01/21 alle ore 17:28:05