Which are the symptoms of Evans Syndrome?

See the worst symptoms of affected by Evans Syndrome here

Symptoms of Evans Syndrome

Evans Syndrome is a rare autoimmune disorder characterized by the simultaneous or sequential development of two or more autoimmune cytopenias, which are conditions involving the destruction of blood cells by the immune system. The two most common cytopenias associated with Evans Syndrome are autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP).

Autoimmune Hemolytic Anemia (AIHA):

AIHA occurs when the immune system mistakenly attacks and destroys red blood cells, leading to a decrease in their numbers. This can result in symptoms such as fatigue, weakness, pale skin (pallor), shortness of breath, rapid heartbeat, and jaundice (yellowing of the skin and eyes). Individuals with AIHA may also experience dark urine due to the presence of hemoglobin breakdown products.

Immune Thrombocytopenia (ITP):

ITP is characterized by a low platelet count, which can lead to abnormal bleeding or bruising. Common symptoms include easy bruising, petechiae (small red or purple spots on the skin), prolonged bleeding from cuts or injuries, nosebleeds, and excessive bleeding during menstruation. In severe cases, individuals with ITP may experience bleeding in the digestive tract or brain.

Other Symptoms:

In addition to AIHA and ITP, individuals with Evans Syndrome may develop other autoimmune cytopenias, such as autoimmune neutropenia (low white blood cell count) or autoimmune lymphoproliferative syndrome (ALPS). These conditions can further weaken the immune system and increase the risk of infections.

Furthermore, individuals with Evans Syndrome may experience general symptoms of autoimmune disorders, including fatigue, malaise, joint pain, muscle aches, and swollen lymph nodes. Some individuals may also develop symptoms related to the underlying cause of Evans Syndrome, such as an enlarged spleen or liver.

Diagnosis and Treatment:

Diagnosing Evans Syndrome can be challenging due to its rarity and the overlapping symptoms with other autoimmune disorders. A thorough medical history, physical examination, and various laboratory tests are typically conducted to confirm the diagnosis.

Treatment for Evans Syndrome aims to manage the autoimmune cytopenias and alleviate symptoms. The approach may involve a combination of medications, such as corticosteroids, immunosuppressants, intravenous immunoglobulin (IVIG), and rituximab. In severe cases, splenectomy (surgical removal of the spleen) may be considered.

Prognosis:

The prognosis for individuals with Evans Syndrome varies depending on the severity of the condition and the response to treatment. Some individuals may experience spontaneous remission, while others may require long-term management of their autoimmune cytopenias. Regular follow-up appointments with healthcare providers are essential to monitor blood counts, manage symptoms, and address any potential complications.

Diseasemaps

For me, the worst symptoms have been related to my experience of severe haemolytic anaemia. When my haemoglobin was very low, I experienced constant breathlessness and tachycardia. I got chest pain as well and this had to be watched carefully as with the heart working so hard for so long, there was a substantial risk of heart attack. I was unable to so much as raise an arm for without my heart rate going up. I was unable to sit up or walk because I had so little blood that I'd faint if I tried. It was completely debilitating. I needed nurses for everything and couldn't even get up to go to the toilet. My urine also turned the colour of strong black espresso and I didn't smell pleasant either because dead red cells smell like a dead animal. I also turned yellow from the cell death too.

Posted Mar 4, 2017 by Ceara 1000