EurekaMag.com logo
+ Site Statistics
References:
53,517,315
Abstracts:
29,339,501
+ Search Articles
+ Subscribe to Site Feeds
EurekaMag Most Shared ContentMost Shared
EurekaMag PDF Full Text ContentPDF Full Text
+ PDF Full Text
Request PDF Full TextRequest PDF Full Text
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter
Follow on Google+Follow on Google+
Follow on LinkedInFollow on LinkedIn

+ Translate

Evans syndrome in childhood: pathophysiology, clinical course, and treatment



Evans syndrome in childhood: pathophysiology, clinical course, and treatment



American Journal of Pediatric Hematology/Oncology 10(4): 330-338



Evans syndrome is defined as the simultaneous or sequential occurrence of Coombs' positive hemolytic anemia and immune thrombocytopenia without known underlying etiology. Ten cases of Evans syndrome were seen at our hospital over the past decade; three patients died. Two cases are described in detail and demonstrate the chronic, refractory nature of this condition. Most patients have required corticosteroid therapy and splenectomy, but further therapy (e.g., intravenous gammaglobulin, danazol, cyclophosphamide) is usually necessary because of recurrent autoimmune hemolytic anemia, thrombocytopenia, or both. There is a substantial risk for development of other autoimmune problems and hypogammaglobulinemia. A number of defects in humoral immunity have been described in Evans syndrome; different antibodies are directed against platelets and red blood cells. Cellular immunity is probably abnormal, but a distinct pattern of immunoregulatory disturbance has not been identified.

(PDF 0-2 workdays service: $29.90)

Accession: 040075458

Download citation: RISBibTeXText

PMID: 3071168



Related references

Epidemiology, pathophysiology, clinical characteristics and management of childhood cardiorenal syndrome. World Journal of Nephrology 1(1): 16-24, 2012

Evans syndrome in childhood. Journal of Pediatrics 97(5): 754-758, 1980

Serious organ damage and intractable clinical conditions in rheumatic and connective tissue disease--progress in pathophysiology and treatment. Topics: II. Clinical conditions special attention needed to be paid to; 4. Macrophage activating syndrome and hemophagocytic syndrome. Nihon Naika Gakkai Zasshi. Journal of the Japanese Society of Internal Medicine 102(10): 2639-2644, 2014

Evans' syndrome and immunologic pancytopenia in childhood. Sangre 33(5): 383-386, 1988

Rituximab therapy for childhood Evans syndrome. Haematologica 92(12): 1691-1694, 2007

Syndrome X: background, clinical aspects, pathophysiology & treatment. Giornale Italiano di Cardiologia 24(6): 779-790, 1994

Kawasaki syndrome: Pathophysiology, clinical picture, and treatment. Infusionstherapie und Transfusionmedizin 23(SUPPL 4): 51-54, 1996

Pathophysiology, clinical consequences, and treatment of tumor lysis syndrome. American Journal of Medicine 116(8): 546-554, 2004

Alcohol withdrawal syndrome clinical features pathophysiology and treatment. Miller, N S (Ed ) Comprehensive Handbook Of Drug And Alcohol Addiction Xxi+1331p Marcel Dekker, Inc : New York, New York, Usa; Basel, Switzerland Illus 1091-1112, 1991

Thrombocytopenia in the antiphospholipid syndrome: pathophysiology, clinical relevance and treatment. Annales de Medecine Interne 147 Suppl 1: 24-27, 1996

Clinical spectrum, pathophysiology and treatment of the Wiskott-Aldrich syndrome. Current Opinion in Hematology 18(1): 42-48, 2014

Therapeutic effect of Rituximab in childhood immune thrombocytopenia and Evans syndrome. Pediatric Research 51(4 Part 2): 241A, April, 2002

Complex regional pain syndrome--pathophysiology, clinical presentation and treatment. Reumatizam 60(2): 67-80, 2015

Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies. Neurosurgical Focus 38(2): E14-E14, 2015

Shock lung. Pathophysiology and clinical treatment of the syndrome - a survey of literature. Casopis Lekaru Ceskych 115(16): 473-476, 1976