Evans Syndrome: A case report

Porcaro, Federica; Valenzise, Mariella; Candela, G; Chiera, Fernanda; Corica, Domenico; Pitrolo, Elda; Santucci, Simona; Romeo, Marika; Nigro, S Cavallaro; Zirilli, Giuseppina

doi:10.4081/pmc.2014.91

Cited by 6 publications

(13 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Evans syndrome is defined as the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). 1,2 There is no preferential distribution of this disease by age, gender, or ethnic group. Its chronic course is characterized by recurrent relapses and remissions.…”

Section: Discussionmentioning

confidence: 99%

“…Its chronic course is characterized by recurrent relapses and remissions. 2 The etiology is unknown and immune dysregulation may be involved in the pathogenesis of the disease. Constitutive increase in production of interleukin-10 and Interferon-Gamma may lead to activation of autoreactive antibody-producing B cells which inturn produces antibodies against different lineages of blood cells.…”

Section: Discussionmentioning

confidence: 99%

“…These signs may be chronic or intermittent and in same case may occur during acute exacerbations. 2 The appearance of the second cytopenia may occur months to years after the first immune cytopenia and may delay diagnosis. Neutropenia may be present in 55% of the cases.…”

Section: Discussionmentioning

confidence: 99%

“…Neutropenia may be present in 55% of the cases. 2,6 Total serum immunoglobulin levels IgG, IgM levels may be decreased. Auto antibodies against RBC is diagnostic of this syndrome but presence of antiplatelet antibodies may be inconsistent.…”

Section: Discussionmentioning

confidence: 99%

“…Evans syndrome is defined as the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). 1,2 Most of them present with severe anemia and moderate thrombocytopenia while neutropenia is associated in some cases. Associated laboratory parameters include unconjugated hyperbilirubinemia, decreased total serum IgG and IgM levels, presence of non-cross reacting auto antibodies against erythrocytes, platelets and in some cases against neutrophils.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Evans syndrome with severe thrombocytopenia: a rare presentation

Kamalamma¹,

Ml²,

Badam³

et al. 2016

Int J Contemp Pediatr

View full text Add to dashboard Cite

Evans syndrome is a rare disease characterized by the sequential or simultaneous presence of autoimmune haemolytic anemia and immune thrombocytopenia with a chronic relapsing and remitting course. Most of them present with severe anemia and moderate thrombocytopenia while neutropenia is associated in some cases. Associated laboratory parameters include unconjugated hyperbilirubinemia, decreased total serum IgG and IgM levels, presence of noncross reacting auto antibodies against erythrocytes, platelets and in some cases against neutrophils. Management of evans syndrome includes glucocorticosteroids as the first line therapy and IVIG as second line treatment. Resistant cases are treated with cyclosporine, mycophenolate mofetil, vincristine, danazol, rituximab (monoclonal anti-CD20 antibody), alemtuzumab (humanized monoclonal anti-CD52 antibody). Autogenic or allogenic hematopoietic stem cell transplantation (HSCT) is the only curative option available. Here we are presenting an adolescent boy with Evans syndrome who presented with severe thrombocytopenia and moderate degree of haemolytic anemia and responded well to first line oral corticosteroid therapy.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Evans syndrome with severe thrombocytopenia: a rare presentation

Kamalamma¹,

Ml²,

Badam³

et al. 2016

Int J Contemp Pediatr

View full text Add to dashboard Cite

show abstract

Dengue Infection and Its Relationship with Evans Syndrome: A Pediatric Case

Gómez

López

Carreño

et al. 2021

Case Reports in Medicine

View full text Add to dashboard Cite

Dengue is a single-stranded RNA virus belonging to the Flaviviridae family. It is an endemic virus in tropical countries. In Colombia, 4 serotypes are present, and the disease is a burden for public health, social programs, and the economic sectors. The main vector is Aedes aegypti, and most infections are asymptomatic or minimally symptomatic. The hemorrhagic appearances of severe dengue are due to plasma leakage as a result of increased vascular permeability, severe thrombopenia, and hemoconcentration. In 2020, 78,979 cases of dengue were reported in Colombia. 38,836 (49.2%) of them were warning-free signs, 39,246 (49.7%) with warning signs, and 897 (1.1%) of severe dengue. As it is well-known, viral diseases are immune system activators, triggering off a loss of tolerance in it. Dengue is not an exception, and it is able to explain different autoimmune phenomena including macrophage activation. Mechanisms have been described by which an exacerbated response of the disease is triggered through the increase of infected cells, formation of immune complexes, and complement pathway activation, which lead to a cross-reaction of viral antigens with epithelial cells with platelets with subsequent endothelial dysfunction and bleeds. The first description of Evans syndrome was made in 1951 by Robert Evans. This syndrome is characterized by the combination of autoimmune hemolytic anemia, immune thrombocytopenia, and, less common/usual, immune neutropenia. This disease’s etiology is unknown, and the dysregulation of the immune system is among its possibilities. Here, we present the case of an unusual hematological and immunological complication of a patient who developed Evans syndrome during severe dengue, taking into account the concomitantly limited literature available for these two diseases, the need for a broader diagnostic approach, multidisciplinary intervention, and a more complex therapeutic approach.

show abstract

Diagnosis of Evan's syndrome

Angelopoulos¹,

Kirkilesis²,

Kakavia³

et al. 2021

Pan Afr Med J

View full text Add to dashboard Cite

This manuscript concerns the case of a patient hospitalized and diagnosed with Evans syndrome. She was hospitalized with signs of thrombocytopenia induced purpura, petechiae, ecchymosis and anemia. She was successfully treated with corticoids and blood transfusions. Our purpose is to explain her clinical presentation and the exams, we used in order to make the diagnosis of Evans syndrome, which requires great suspicion. Moreover, other diseases causing hemolytic anemia and thrombocytopenia must be excluded. We used laboratory tests (blood samples, Coombs examination and virologic test). Bone marrow examination took place twice. Evans syndrome is an autoimmune disease which is characterized by the coexistence of hemolytic anemia and immune-mediated thrombocytopenia. There is no typical clinical presentation. Its etiology is unknown and its therapy is generally poor. Diagnosis of Evans syndrome is very difficult and requires the exclusion of other diseases causing anemia and thrombocytopenia.

show abstract

Evans Syndrome: A case report

Cited by 6 publications

References 6 publications

Evans syndrome with severe thrombocytopenia: a rare presentation

Evans syndrome with severe thrombocytopenia: a rare presentation

Dengue Infection and Its Relationship with Evans Syndrome: A Pediatric Case

Diagnosis of Evan's syndrome

Contact Info

Product

Resources

About