RESUMO
Evans syndrome is a condition in which autoimmune-mediated red blood cells and platelet destruction happens consecutively. It may be associated with a reduction in neutrophil count as a result of immune neutropenia. No sex preference is known and it presents in all ages and any ethnic cohort. Generally, this syndrome tends to be chronic and is characterised by remission and exacerbation. We document a case of the immune-mediated disease associated with Epstein-Barr virus infection in an 8-year-old boy from eastern Sudan who presented with both immune thrombocytopenia purpura and autoimmune haemolytic anaemia. Complete blood count and peripheral blood picture revealed features consistent with immune haemolytic anaemia (rouleaux formation and spherocytes) and thrombocytopenia. Direct anti-human globulin test and indirect anti-human globulin test were positive. Evans syndrome is a potentially life-threatening condition due to the concomitant existence with antiplatelet and anti-erythrocyte antibodies distinguished by a positive antiglobulin test and possibly linked to other autoimmune or lymphoproliferative diseases.
RESUMO
Congenital insensitivity to pain with anhidrosis (CIPA), also called hereditary sensory and autonomic neuropathy type IV (HSAN-IV), is an extremely rare autosomal recessive disease. It is characterized by insensitivity to pain, inability to sweat, episodes of hyperpyrexia, and intellectual disability. These factors render the affected persons to repeatedly injure and traumatise themselves to the degree that they become disabled. No specific treatment to the moment, but it needs multidisciplinary approach, and certain life adaptations and education. Here we report 4 unrelated Sudanese children affected with this rare neurological disorder, to raise the awareness on this rare disease, reflecting its spectrum, and the challenges which patients and their families face, especially when living in a hot country.
