- immune thrombocytopenia and autoimmune hemolytic anemia
- occurs in individuals of all ages. In a 1997 survey of North American
pediatric hematologists, the median reported age at diagnosis was 7.7 years
(range 0.2-26.6 y)
- an uncommon but not rare condition; its exact frequency is unknown.
Familial occurrence is rare.
- The etiology of Evans syndrome remains speculative. Autoantibodies are
directed against specific antigens specific to red cells, platelets, or
neutrophils, but they do not cross react.
- has a chronic, relapsing, and sometimes fatal course.
- Evans syndrome is a diagnosis of exclusion. Confounding disorders, such as
infections, rheumatologic diseases, malignancies can present with autoimmune
cytopenias, must be ruled out.
History: In a national survey, thrombocytopenia was the presenting
feature in 76% of patients with Evans syndrome, and anemia was the presenting
feature in 67% of these patients. In addition, 24% of patients had neutropenia
and 14% had pancytopenia.
- The CBC and reticulocyte count show anemia, thrombocytopenia, neutropenia,
or combined cytopenias; the reticulocyte count increases if the patient has
- In Evans syndrome, the direct antiglobulin result (ie, Coombs test result)
- A variety of antibodies directed against RBCs and platelets (eg,
antierythrocyte, antineutrophil, antiplatelet antibodies) occur in association
with Evans syndrome.
- Lupus antibody and antinuclear antibody (ANA) tests are used to detect SLE.
- Perform T- and B-cell function tests for quantitative immunoglobulins to
evaluate for hypogammaglobulinemia and perturbations in T-cell numbers.
- Bone marrow aspiration helps reveal aplastic anemia or an infiltrative
disorder. Bone marrow aspiration may be indicated when patients initially
present with pancytopenia.
- Prednisone therapy often effectively controls acute episodes, though
relapses may be frequent when patients are weaned off prednisone.
- Intravenous immunoglobulin (IVIG) may help patients who are dependent on
- Other therapies that were effective in small series were danazol,
cyclosporine, azathioprine, cyclophosphamide, and vincristine.
- Additional therapies are splenectomy and hematopoietic stem cell
- Splenectomy may improve blood counts and reduce steroid dependence, but
relapses are common.
- Children with Evans syndrome appear to have a higher risk of
postsplenectomy sepsis, especially children with pancytopenia.
eMedicine.com. Evans Syndrome. Last Updated: June 15, 2005