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.

Rheumatoid arthritis

Inflammatory changes in the synovial membranes and articular structures with widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, as well as atrophy and rarefaction of bony structures.


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PMID (PMCID)
23321502
 FEMALE Middle Aged
Simultaneous romiplostin, eltrombopag, and prednisone were successful in severe thrombocytopenia of Evans syndrome refractory to hydrocortisone, splenectomy, intravenous IgG, and rituximab.
Ruiz-Arguelles GJ, Ruiz-Delgado GJ, Velazquez-Sanchez-de-Cima S, Zamora-Ortiz G.
Hematology. 2013;18(3):175-7.
A 58-year-old woman presented with rheumatoid arthritis-associated Evans syndrome (simultaneous autoimmune hemolytic anemia and autoimmune thrombocytopenic purpura); she was treated unsuccessfully with steroids, romiplostin, rituximab, immunoglobulin G, and splenectomy.
1925397
 MALE Middle Aged
[Evans' syndrome caused by D-penicillamine in rheumatoid arthritis. Value of the corticoids-danazol combination].
Masson C, Bregeon C, Ifrah N, Berton V, Housseau F, Renier JC.
Rev Rhum Mal Osteoartic. 1991;58(7):519-22.
[Evans' syndrome caused by D-penicillamine in rheumatoid arthritis.