Evans syndrome is an autoimmune disease in which an individual’s antibodies attack their own red blood cells and platelets. Symptoms of Evans syndrome include breathlessness, fatigue, jaundice, and dark urine. Laboratory findings consistent with the diagnosis of Evans syndrome include anemia, positive direct coombs test, and positive antineutrophil antibody. Evans syndrome must be differentiated from acquired thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, Kasabach-Merritt syndrome, fibromatosis, fibrosarcoma, nodular fasciitis and myxoid neurofibroma. Pharmacologic medical therapies for Evans syndrome include prednisone, intravenous immunoglobulin, and rituximab. Surgery is not the firstline treatment option for patients with Evans syndrome. Splenectomy is usually reserved for patients who are unresponsive to treatment.
What are the symptoms of Evans syndrome?
Symptoms of carriers are as follows:
- Feeling faint
- Shooting pains
- Faster heart rate
- Ulcers from mucous membrane
In autoimmune hemolytic anemia the following may be found:
- Pale skin color
- Shortness of breath
- Rapid heartbeat
- Yellow/jaundice colored eyes and skin
- Dark urine
What causes Evans syndrome?
Who is at highest risk?
- 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.
- Peripheral smear – A peripheral smear of the blood may reveal evidence of red blood cell destruction or reticulocytosis. Reticulocytes are immature red blood cells and are usually abundant in Evan’s syndrome where there is a need to replace ongoing losses. 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.
- A Coombs test is used to detect the presence of antibodies against the red blood cell and is usually positive in immune mediated hemolysis.
- Other antibodies may occur directed against neutrophils and lymphocytes,
When to seek urgent medical care?
- Call your health care provider if symptoms of Evans syndrome develop.
- Pharmacologic medical therapies for Evans syndrome include prednisone, intravenous immunoglobulin, and rituximab. Surgery is not the firstline treatment option for patients with Evans syndrome. Splenectomy is usually reserved for patients who are unresponsive to treatment.
Where to find medical care for Evans syndrome?
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- Pegels JG, Helmerhorst FM, van Leeuwen EF, van de Plas-van Dalen C, Engelfriet CP, von dem Borne AE (1982). “The Evans syndrome: characterization of the responsible autoantibodies”. Br. J. Haematol. 51 (3): 445–50. doi:10.1111/j.1365-2141.1982.tb02801.x. PMID 7104228.CS1 maint: Multiple names: authors list (link)