Evans syndrome is a rare autoimmune disorder where the immune system attacks red blood cells, white blood cells, and platelets. It was first described in 1951 and causes hemolytic anemia from red blood cell destruction and thrombocytopenia from low platelet counts. Symptoms vary depending on which blood cells are affected but may include weakness, fatigue, bruising, or infections. Diagnosis involves blood tests and treatment depends on severity but can include blood transfusions, steroids, IVIG, immunosuppressants, rituximab, or stem cell transplant.

1. Evans Syndrome
By,
Ms. Ekta S Patel,
Assistant Professor.

2. “
▪ Evans syndrome is an autoimmune
disease in which an
individual's immune system attacks
their own red blood cells, white
blood cells, and/or platelets.
▪ These events may occur
simultaneously or one may follow
on from the other.

3. “▪ Evans syndrome was
first described in the
medical literature in
1951 by Dr. Robert
Evans and associates

4. ▪ Affected people often
experience thrombocytopenia (too few platelets)
and Coombs' positive hemolytic
anemia (premature destruction of red blood cells).
▪ The best treatment options for Evans syndrome
depend on many factors, including the severity of
the condition; the signs and symptoms present;
and each person's response to certain therapies

5. Definition:
▪ Evans syndrome is a rare chronic hematologic
disorder characterized by the simultaneous or
sequential association of autoimmune hemolytic
anemia with immune thrombocytopenic purpura and
occasionally autoimmune neutropenia, in the
absence of a known underlying etiology.

6. Etiology:
▪ The exact cause of Evans syndrome is not known.
▪ Disorders of the immune system like Evans
syndrome occur when the immune system
produces antibodies that mistakenly attack
healthy tissue, specifically red blood
cells, platelets, and white blood cells.

7. ▪ Evans syndrome may occur in combination with another
disorder as a secondary condition.
▪ autoimmune lymphoproliferative syndrome(ALPS),
▪ lupus,
▪ antiphospholipid syndrome,
▪ Sjogren (Show-grins) syndrome,
▪ common variable immunodeficiency,
▪ IgA deficiency,
▪ certain lymphomas,
▪ chronic lymphocytic leukemia.

8. Clinical Manifestation:
▪ The signs and symptoms of Evans syndrome vary
from person to person and largely depend on
which type(s) of blood cells are affected (i.e. red
blood cells, platelets, and/or white blood cells).

9. ▪ If a person does not have enough healthy red
blood cells (anemia), they may experience;
▪ Weakness,
▪ Fatigue,
▪ Paleness,
▪ Light-headedness,
▪ Shortness of breath, or rapid heartbeat.

10. ▪ Low platelets (thrombocytopenia) can cause
▪ Easy or unexplained bruising;
▪ Red or purple spots on the skin (petechiae);
▪ Prolonged bleeding from small cuts
▪ Purpura.

11. ▪ People with a low white blood cell count
(neutropenia) may be;
▪ More susceptible to infections,
▪ May experience symptoms such as fever
▪ Sores inside the mouth.

12. ▪ Many people with Evans syndrome go through
periods of remission in which the signs and
symptoms of the condition temporarily disappear
or become less severe (usually induced
temporarily by treatment).

13. ▪ This table lists symptoms that people with this
disease may have.
▪ This information comes from a database called
the Human Phenotype Ontology (HPO) .

15. Diagnostic Evaluation:
▪ Various blood tests,
▪ CBC
▪ Reticulocyte count
▪ Coombs test
▪ Test for antineutrophil and antiplatelet antibody

16. ▪ Lupus antibody and antinuclear antibody (ANA)
test
▪ Measurements of serum immunoglobulin
▪ Flow cytometry of blood sample
▪ Gene mutation study.
▪ Bone marrow aspiration,

17. Treatment:
▪ Treatment for Evans syndrome depends on many
factors, including the severity of the condition;
the signs and symptoms present; and each
person's response to certain therapies.

18. ▪ For example, people who need to be hospitalized
due to severe anemia or thrombocytopenia are
often treated with blood transfusions followed
by therapy with corticosteroids or intravenous
(IV) immune globulin.

19. ▪ Other treatment options include
immunosuppressive drugs.
▪ Most affected individuals respond to these
treatments; however, relapse is frequent.

20. ▪ In people who do not respond to standard
treatments, therapy with rituximab may be
considered.
▪ Some people with Evans syndrome respond well
to rituximab treatment and experience an
extended period of remission, while others have
little to no response

21. ▪ The role of splenectomy in treating Evans
syndrome is not clearly established.
▪ While splenectomy may lead to immediate
improvement, relapses are common and usually
occur within 1-2 months after the procedure.

22. ▪ For cases that are very severe and difficult to
treat, a stem cell transplant may be used to
provide a long-term cure.
▪ Autologous and allogeneic stem
cell transplantation have been used in a small
number of patients with mixed results.