Evans syndrome

A rare chronic hematologic disorder characterized by the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA; a disorder in which auto-antibodies are directed against red blood cells causing anemia of varying degrees of severity) with immune thrombocytopenic purpura (ITP; a coagulation disorder in which auto-antibodies are directed against platelets causing hemorrhagic episodes) and occasionally autoimmune neutropenia, in the absence of a known underlying etiology.

Immunodeficiency

Failure of the immune system to protect the body adequately from infection, due to the absence or insufficiency of some component process or substance.


Total: 3

                      


(per page)
PMID (PMCID)
25414442
MIXED_SAMPLE Child
Early-onset Evans syndrome, immunodeficiency, and premature immunosenescence associated with tripeptidyl-peptidase II deficiency.
Stepensky P, Rensing-Ehl A, Gather R, Revel-Vilk S, Fischer U, Nabhani S, Beier F, Brummendorf TH, Fuchs S, Zenke S, Firat E, Pessach VM, Borkhardt A, Rakhmanov M, Keller B, Warnatz K, Eibel H, Niedermann G, Elpeleg O, Ehl S.
Blood. 2015;125(5):753-61.
Early-onset Evans syndrome, immunodeficiency, and premature immunosenescence associated with tripeptidyl-peptidase II deficiency.
19127568
FEMALE Infant
Severe Evans syndrome with multi-system involvement is a distinct immunodeficiency disorder.
Jubinsky PT, Moulton T, Tewari P, Short MK.
Pediatr Blood Cancer. 2009;52(5):659-61.
These features place the patient at the most severe portion of the Evans syndrome spectrum, and suggest that this case may represent a rare, new immunodeficiency disorder.
19127568
FEMALE Infant
Severe Evans syndrome with multi-system involvement is a distinct immunodeficiency disorder.
Jubinsky PT, Moulton T, Tewari P, Short MK.
Pediatr Blood Cancer. 2009;52(5):659-61.
Severe Evans syndrome with multi-system involvement is a distinct immunodeficiency disorder.