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.

Nephrotic syndrome

Nephrotic syndrome is a collection of findings resulting from glomerular dysfunction with an increase in glomerular capillary wall permeability associated with pronounced proteinuria. Nephrotic syndrome refers to the constellation of clinical findings that result from severe renal loss of protein, with Proteinuria and hypoalbuminemia, edema, and hyperlipidemia.


Total: 2

                      


(per page)
PMID (PMCID)
23590417
MIXED_SAMPLE Child
Variable presentation of primary immune deficiency: two cases with CD3 gamma deficiency presenting with only autoimmunity.
Tokgoz H, Caliskan U, Keles S, Reisli I, Guiu IS, Morgan NV.
Pediatr Allergy Immunol. 2013;24(3):257-62.
However, Evans syndrome, autoimmune hepatitis, and nephrotic syndrome have not been reported in previous cases.
23590417
MIXED_SAMPLE Child
Variable presentation of primary immune deficiency: two cases with CD3 gamma deficiency presenting with only autoimmunity.
Tokgoz H, Caliskan U, Keles S, Reisli I, Guiu IS, Morgan NV.
Pediatr Allergy Immunol. 2013;24(3):257-62.
Here, we present two siblings from non-consanguineous family with autoimmunity including Evans syndrome, autoimmune hepatitis, nephrotic syndrome, and Hashimoto's thyroiditis and with no previous history of infections.