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RESEARCH PRODUCT
Evans’ Syndrome: From Diagnosis to Treatment
Marc MichelBernard BonnotteSylvain AudiaNatacha GrienayMorgane Mouniersubject
Pediatricsmedicine.medical_specialtyEvans syndromemedicine.medical_treatmentSplenectomylcsh:MedicineDiseaseReview03 medical and health sciences0302 clinical medicinehemic and lymphatic diseasesMedicineCytopeniabusiness.industryMyelodysplastic syndromeslcsh:RGeneral Medicinemedicine.diseaseEvans’ syndromeimmune thrombocytopenia030220 oncology & carcinogenesisConcomitantAutoimmune neutropeniaRituximabautoimmune haemolytic anaemiabusiness030215 immunologymedicine.drugdescription
Evans’ syndrome (ES) is defined as the concomitant or sequential association of warm auto-immune haemolytic anaemia (AIHA) with immune thrombocytopenia (ITP), and less frequently autoimmune neutropenia. ES is a rare situation that represents up to 7% of AIHA and around 2% of ITP. When AIHA and ITP occurred concomitantly, the diagnosis procedure must rule out differential diagnoses such as thrombotic microangiopathies, anaemia due to bleedings complicating ITP, vitamin deficiencies, myelodysplastic syndromes, paroxysmal nocturnal haemoglobinuria, or specific conditions like HELLP when occurring during pregnancy. As for isolated auto-immune cytopenia (AIC), the determination of the primary or secondary nature of ES is important. Indeed, the association of ES with other diseases such as haematological malignancies, systemic lupus erythematosus, infections, or primary immune deficiencies can interfere with its management or alter its prognosis. Due to the rarity of the disease, the treatment of ES is mostly extrapolated from what is recommended for isolated AIC and mostly relies on corticosteroids, rituximab, splenectomy, and supportive therapies. The place for thrombopoietin receptor agonists, erythropoietin, immunosuppressants, haematopoietic cell transplantation, and thromboprophylaxis is also discussed in this review. Despite continuous progress in the management of AIC and a gradual increase in ES survival, the mortality due to ES remains higher than the ones of isolated AIC, supporting the need for an improvement in ES management.
year | journal | country | edition | language |
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2020-11-01 | Journal of Clinical Medicine |