Evan’s Syndrome is a combination of two conditions: autoimmune hemolytic anemia and autoimmune thrombocytopenia purpura. Autoimmune hemolytic anemia is a condition in which there are low levels of iron in the body due to the destruction of the red blood cells that normally carry oxygen. Autoimmune thrombocytopenia is revealed by a low level of platelets in the blood due to their destruction in the circulation. Platelets are a component of blood that is responsible for creating clots in the body to heal wounds.

Those Affected

The incidence of Evan’s Syndrome is not precisely known. The syndrome is reported to be a complication affecting 4-10% of those persons with a particular type of thrombocytopenia known as autoimmune thrombocytopenia purpura. The syndrome is more prevalent in children than in adults.

Signs and Symptoms

The signs and symptoms of Evan’s Syndrome will be a combination of the signs and symptoms of the two underlying conditions. In autoimmune thrombocytopenia purpura the following may be found: Bleeding of skin or mucus lined areas of the body. This may show up as bleeding in the mouth, or purpuric rashes (look almost like bruises), or tiny red dots on the skin called petechiae. Laboratory results will show low levels of platelets

In autoimmune hemolytic anemia the following may be found: Fatigue Pale skin color Shortness of breath Rapid heartbeat Dark urine

Possible Causes

The cause of the signs and symptoms of Evan’s Syndrome are directly related to the low levels of red blood cells (RBC) and platelets in the blood. These low levels are a result of circulating antibodies that bind to the blood cells and destroy them. Antibodies are made under normal conditions against foreign substances in the body and are therefore very useful in warding off infection. In conditions that are referred to as “autoimmune” the body makes antibodies against itself. In the case of Evan’s Syndrome, it is not currently known what triggers this reaction to happen.

Diagnosis

The diagnosis of Evan’s Syndrome is based primarily on laboratory findings, as well as the corresponding physical signs and symptoms. A complete blood count (CBC) will confirm the presence of anemia and low platelets. Additional studies may include a peripheral smear of the blood which may reveal evidence of red blood cell destruction or reticulocytosis, and a coombs test. Reticulocytes are immature red blood cells and are usually abundant in Evan’s syndrome where there is a need to replace ongoing losses. A coombs test is used to detect the presence of antibodies against the RBC and is usually positive. There are also distinct shapes to certain cells that may be found when a sample of the patient’s blood is viewed under a microscope. In patients with Evan’s syndrome the red blood cells may appear small and globular shaped (then called spherocytes) but will not be fragmented.

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Solving the Puzzle of Hemolysis: A Critical Role for the Laboratory
From Clinical Laboratory Science, 7/1/04 by McKenzie, Shirlyn B

This case concerns a 60-year-old woman who was seen in the emergency room for flu-like symptoms, dizziness, weakness, and cough. She reported dark stools and several days of dark colored urine. Previous history revealed suspected hemolytic anemia and mild thrombocytopenia. At admission, her hemoglobin was 5.9 g/dL and platelet count was 100 x 10^sup 9^/L. Evidence of hemolysis included: total bilirubin 1.6 mg/ dL, lactate dehydrogenase 850 U/L, and a positive DAT with IgG and C3. She received four units of packed red blood cells and her hemoglobin stabilized at about 10 g/dL. Upon outpatient follow-up, the complete blood count showed a worsening of the cytopenias with a hemoglobin of 7.7 g/dL and platelet count of 60 x 10^sup 9^/L. The differential showed many abnormalities including marked polychromasia, nucleated red blood cells, basophilic stippling, schistocytes, Pappenheimer bodies, and Howell-Jolly bodies. Of particular interest was phagocytosis of platelets by neutrophils. The reticulocyte count was 37%. The diagnosis of Evan's syndrome, a severe thrombocytopenia with warm autoimmune hemolytic anemia was made. This syndrome is diagnosed after other causes of progressive cytopenias have been ruled out including myelodysplasia, malignancy, and liver and kidney dysfunction. There is no definitive treatment.

Shirlyn B McKenzie PhD CLS(NCA), Brandon Hiller, University of Texas Health Science Center at San Antonio, Department of Clinical Laboratory Sciences, San Antonio TX.

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