Evans syndrome: a study of six cases with review of literature.
Evans syndrome is an uncommon condition characterised by simultaneous or sequential development of immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA) with a positive direct antiglobulin test (DAT) in the absence of a known underlying aetiology. The great majority of patients with Evans syndrome have a chronic relapsing course despite treatment, which is associated with significant morbidity and mortality. We reviewed the clinical and laboratory features of six patients with Evans syndrome. All patients had thrombocytopenia, bleeding symptoms and haemolytic anaemia with positive direct Coombs test at presentation. We discuss the aetiopathogenic, clinical, therapeutic and natural history of Evans syndrome.
Dhingra
Kajal Kiran
KK
Department of Pathology, Maulana Azad Medical College and Lok Nayak Hospital, Bahadur Shah Zafar Marg, New Delhi 110002, India.
Jain
Deepali
D
Mandal
Shramana
S
Khurana
Nita
N
Singh
Tejinder
T
Gupta
Naresh
N
D000744:Anemia, Hemolytic, Autoimmune / Q000209:etiology / Q000473:pathology / Q000628:therapy; D003298:Coombs Test; D006801:Humans; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000209:etiology / Q000473:pathology / Q000628:therapy; D013577:Syndrome