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.

Neutropenia

An abnormally low number of neutrophils in the peripheral blood.


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PMID (PMCID)
28680366
(5496165)
 OTHER
Fatal pulmonary embolism following splenectomy in a patient with Evan's syndrome: case report and review of the literature.
Monga V, Maliske SM, Perepu U.
Thromb J. 2017;15:18.
Evans syndrome (ES) is a rare disease characterized by simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia.
21772952
(3133497)
 OTHER
Effectiveness of cyclosporine and mycophenolate mofetil in a child with refractory evans syndrome.
Farruggia P, Macaluso A, Tropia S, Genova S, Paolicchi O, Di Marco F, D'Angelo P.
Pediatr Rep. 2011;3(2):e15.
Evans Syndrome is a rare autoimmune disease consisting of hemolytic anemia, thrombocytopenia and/or neutropenia.
9519776
 FEMALE Middle Aged
Induction of clinical remission in T-large granular lymphocyte leukemia with cyclosporin A, monitored by use of immunophenotyping with Vbeta antibodies.
Brinkman K, van Dongen JJ, van Lom K, Groeneveld K, Misere JF, van der Heul C.
Leukemia. 1998;12(2):150-4.
A 54-year-old woman presented with a severe autoimmune anemia, thrombocytopenia, neutropenia (Evans' syndrome), and CD8+ lymphocytosis, without signs of lymphadenopathy or splenomegaly.
8616018
 FEMALE Adult
Autoantibody against a 58 kD molecule in a patient with neutropenia and NK cell deficiency.
Witte T, Bode FM, Hammer M, Deicher H, Schmidt RE.
Br J Haematol. 1996;92(3):565-70.
A patient with Evans' syndrome is described who developed intermittently an immunocytopenia consisting of autoimmune neutropenia and natural killer (NK) cell deficiency.
315788
 MALE
Immune pancytopenia.
Daneshbod-Skibba G, Shahidi NT, Becker GA, Martin J.
Br J Haematol. 1979;43(1):7-14.
These findings indicate that T-lymphocytes may play a role in the pathogenesis of neutropenia in immune pancytopenia.