Causes and risk factors
The exact cause and mechanism of Evans syndrome is not known. However, studies have shown that there exists some abnormality in T- cells. Decrease in T helper cells and increase in the T suppressor lymphocytes have been related to it. Evans syndrome can also be present with certain other conditions like systemic lupus erythematosus. Certain other autoimmune disorders can also be associated to it like the autoimmune lymphoproliferative or antiphospholipid syndrome or other immunodeficient diseases.
Clinical presentation:
An individual can suffer from thrombocytopenia initially and later the signs and symptoms of hemolytic anemia can be seen. In most of the cases these conditions occur simultaneously. Those with minimal changes in RBC can remain asymptomatic for a long while, others can present with complaints of fatigue and tiredness. Due to inadequate number of blood cells, the circulation is also hampered. As a result, the skin becomes pale; the extremities are cold to touch. Difficulty in breathing, pain in chest, pain in abdomen, etc., are other symptoms seen. Due to lack of blood supply, the heart has to overwork. Simultaneously, the other parts of the body are deprived of adequate amount of oxygen, which in turn gives rise to a variety of complaints. In severe cases, yellowish discoloration of the skin and mucous membrane, fever, chills, prostration, and even shock can be seen. The urine becomes dark red colored due to presence of free hemoglobin in the blood.
Immune thrombocytopenia is revealed when the patient complaints of easy bruising along with bleeding. Reddish purple spots are seen on skin. Blood is present in stool and urine. Small wounds bleed easily or bleeding can occur for a prolonged time. Epistaxis along with bleeding in other parts of the body is also seen. Profound hemorrhages and infections are the common complications which can occur.
Investigations:
The signs and symptoms of the patient are taken into consideration. Diagnosis is also confirmed by ruling out other disorders. Hematological test helps in confirmation of the disease. A complete blood count is advised. Liver function test can also be done. A positive direct antiglobulin test (Coombs Tests) confirms the diagnosis.
Treatment:
In spite of treatment, there are great chances of recurrence of complaints, hence prognosis is poor. The main line of treatment consists of administration of immunocompressive therapy along with intravenous administration of immunoglobulin. Splenectomy can also be done in cases of any spleen disorders. Hematopoietic stem cell transplantation is needed in severe cases.
Other Modes of treatment:
Certain other modes of treatment can also be helpful in coping up with the symptoms. Taking into consideration the symptoms in a holistic way, homoeopathy can offer a good aid for the relief of the symptoms.
