Evans syndrome (Updated – May 2014.)

Teachey DT, Lambert MP. Diagnosis and management of autoimmune cytopenias in
childhood. Pediatr Clin North Am. 2013 Dec;60(6):1489-511.

“ES is a diagnosis of exclusion, defined by idiopathic autoimmune destruction of multiple cell lineages. Patients with ES tend to have chronic disease and present at a young age (median 7–8 years) with symptoms based on the affected cell lineages. [31,32] Different definitions for ES exist in the literature, making study comparison challenging. [31,32,33,34] Some investigators and clinicians consider any patient with autoimmune disease affecting 2 or more cell lineages as having ES. Others restrict the definition to patients with autoimmune destruction of red cells and platelets with or without neutrophil destruction. Some studies restrict the diagnosis to patients with clinically relevant destruction of multiple lineages, whereas others include patients with ITP and a positive DAT without obvious hemolysis. Patients often present with splenomegaly with or without lymphadenopathy and may have an increased risk of secondary lymphomas. [31,32,34, 35]”

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Dhingra KK, Jain D, Mandal S, Khurana N, Singh T, Gupta N. Evans syndrome: a study of six cases with review of literature. Hematology. 2008 Dec;13(6):356-60.

“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 withEvans 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.”

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Norton A, Roberts I. Management of Evans syndrome. Br J Haematol. 2006 Jan;132(2):125-37.

“Evans syndrome is an uncommon condition defined by the combination (either simultaneously or sequentially) of immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA) with a positive direct antiglobulin test (DAT) in the absence of known underlying aetiology. This condition generally runs a chronic course and is characterised by frequent exacerbations and remissions. First-line therapy is usually corticosteroids and/or intravenous immunoglobulin, to which most patients respond; however, relapse is frequent. Options for second-line therapy include immunosuppressive drugs, especially ciclosporin or mycophenolate mofetil; vincristine; danazol or a combination of these agents. More recently a small number of patients have been treated with rituximab, which induces remission in the majority although such responses are often sustained for <12 months and the long-term effects in children are unclear. Splenectomy may also be considered although long-term remissions are less frequent than in uncomplicated ITP. For very severe and refractory cases stem cell transplantation (SCT) offers the only chance of long-term cure. The limited data available suggest that allogeneic SCT may be superior to autologous SCT but both carry risks of severe morbidity and of transplant-related mortality. Cure following reduced-intensity conditioning has now been reported and should be considered for younger patients in the context of controlled clinical trials.”

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Savaşan S, Warrier I, Ravindranath Y. The spectrum of Evans’ syndrome. Arch Dis Child. 1997 Sep;77(3):245-8.

“Eleven patients (10 boys, one girl) with Evans’ syndrome with a median follow up time of 8.0 years were evaluated retrospectively. Six patients had either persistent hepatosplenomegaly or generalised lymphadenopathy, or both. In five patients, an increase in lymph node and/or spleen size was observed during the exacerbations of cytopenias. Seven patients had quantitative serum immunoglobulin abnormalities at the time of presentation. There were associated systemic manifestations in nine patients. Various forms of treatment were used with mixed results. Four patients died from sepsis and haemorrhage; four had complete recovery–two after splenectomy. These findings show that Evans’ syndrome is a heterogeneous disorder with significant morbidity and mortality. High incidence of quantitative serum immunoglobulin abnormalities, lymphoid hyperplasia, and associated systemic manifestations suggest that Evans’ syndrome may represent a stage of a more broad spectrum, generalised immune dysregulation.”

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Wang WC. Evans syndrome in childhood: pathophysiology, clinical course, and treatment. Am J Pediatr Hematol Oncol. 1988 Winter;10(4):330-8.

“Evans syndrome is defined as the simultaneous or sequential occurrence of Coombs’ positive hemolytic anemia and immune thrombocytopenia without known underlying etiology. Ten cases of Evans syndrome were seen at our hospital over the past decade; three patients died. Two cases are described in detail and demonstrate the chronic, refractory nature of this condition. Most patients have required corticosteroid therapy and splenectomy, but further therapy (e.g., intravenous gammaglobulin, danazol, cyclophosphamide) is usually necessary because of recurrent autoimmune hemolytic anemia, thrombocytopenia, or both. There is a substantial risk for development of other autoimmune problems and hypogammaglobulinemia. A number of defects in humoral immunity have been described in Evans syndrome; different antibodies are directed against platelets and red blood cells. Cellular immunity is probably abnormal, but a distinct pattern of immunoregulatory disturbance has not been identified.”

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Created 12/18/12, updated 05/13/14. 

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