Evans Syndrome: Regarding a Clinical Case

Introduction: Evans syndrome (ES) is a rare hemolytic disorder that can be caused by the simultaneous or sequential association of autoimmune hemolytic anemia and autoimmune thrombocytopenia.

It is a rare disorder and the diagnose needs a sequencial evaluation with exclusion of other diseases.

The average age of presentation in children is around 5.4-7.7 years. The pathophysiology of ES is still unknown and it can be classified as primary or secondary.

The manifestations can include thrombocytopenia, anemia, neutropenia and pancytopenia. Patients can present with bruises, petechiae, purple, pallor, fatigue and even episodes of dizziness.

In a first approach, the blood count is essencial.

Clinical Case: Teenager, 15 years old, male, soccer player, aspiring professional in this category. He presents with scattered bruises and cervical adenomegaly, with no other changes on physical examination.

The result of the blood count showed initial pancytopenia, associated with autoimmune hemolytic anemia.

After a multidisciplinary study, he was diagnosed with primary ES and followed up in a pediatric hematology consultation. He had several periods of exacerbations and remissions, and after 4 years he was discharged from this consultation.

Currently, the hypothesis of ES with associated SLE seems to gain more importance with the recent positivity of antinuclear antibodies.

Conclusion: The objective of this clinical case was to alert to the importance of recognition of this pathology, despite its rarity and the differential diagnosis.

The role of the family doctor in addressing this pathology is noteworthy, not only because of the suspicion of the diagnosis, but also because of the assessment of complaints and symptoms that may suggest secondary etiologies of SE and permanent communication with secondary health care.

It should be noted that the evolution of the disease conditioned changes in the family cycle, also leading to changes in the patient`s life project.