
Health Dictionary
Reliable, easy-to-read patient information on common medical conditions β written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Evans syndrome natural history, complications and prognosis
Introduction
Overview
Overview
If left untreated, patients with Evans syndrome have periods of exacerbation. Common complications of Evans syndrome include thrombocytopenia and autoimmune haemolytic anaemia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.
Natural History
Natural History
- The disease has a chronic course with periods of exacerbation and remissions. The remissions are induced by treatment.
Prognosis
Prognosis
- The prognosis depends on the patientβs response to treatment. Spontaneous remissions of each of the individual component conditions have been reported. If the child responds well to the treatment and the levels of platelets and red blood cells increase, the child can expect to live a normal life. Medications will be needed life long, and laboratory tests will need to be constantly monitored to detect any abnormal changes so that treatment can be adjusted.
- In more serious cases Evans syndrome can reduce life span and may be seriously life threatening.
- Evan’s Syndrome is rare and has a reported mortality rate of just under 18%.
- It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia,[1] with recent research finding that 58% of children with Evans syndrome have CD4-/CD8- T cells which is a strong predictor for having autoimmune lymphoproliferative syndrome.[2]
References
References
- β Wang WC (1988). “Evans syndrome in childhood: pathophysiology, clinical course, and treatment”. The American journal of pediatric hematology/oncology. 10 (4): 330β8. doi:10.1097/00043426-198824000-00013. PMIDΒ 3071168.
- β Teachey DT; Manno CS; Axsom KM; et al. (2005). “Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS)”. Blood. 105 (6): 2443β8. doi:10.1182/blood-2004-09-3542. PMIDΒ 15542578.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2] Ammu Susheela, M.D. [3]
