Evans syndrome with auto‐immune neutropenia

Intracranial Haemorrhage as a Fatal Complication of Evans Syndrome: A Case Report

Nepal Medical Journal ◽

10.37080/nmj.76 ◽

2019 ◽

Vol 2 (2) ◽

pp. 66-69

Author(s):

Olita Shilpakar ◽

Bibek Rajbhandari ◽

Bipin Karki ◽

Umesh Bogati

Keyword(s):

Intracranial Haemorrhage ◽

Early Recognition ◽

Clinical Manifestations ◽

Health Care Facilities ◽

Evans Syndrome ◽

Fatal Complication ◽

Hematologic Disorder ◽

Immune Mediated ◽

Life Threatening ◽

Immune Neutropenia

Evans syndrome is a rare hematologic disorder characterized by the presence of simultaneous or sequential direct Coombs-positive autoimmune hemolytic anemia (AIHA), immune-mediated thrombocytopenia and/or immune neutropenia without any known underlying etiology. Spontaneous intracranial hemorrhage is a rare and life-threatening complication in patients with Evans syndrome and very few cases have been reported to date. We report a case of a thirty-two- year-old female with intracranial haemorrhage with underlying Evans syndrome who presented with the clinical manifestations of headache, vomiting and altered sensorium and succumbed to the fatal complication despite resuscitative measures. This also emphasizes the importance of early recognition of symptoms and immediate presentation to health care facilities for aggressive management of the patient.

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A Female with Evans Syndrome Presented with Pancytopenia

Journal of Enam Medical College ◽

10.3329/jemc.v10i2.53537 ◽

2021 ◽

Vol 10 (2) ◽

pp. 114-117

Author(s):

Md Rezaul Karim Chowdhury ◽

Md Haroon Ur Rashid ◽

Md Mahbub Hossain ◽

Shafayet Hossain Riyan

Keyword(s):

Oral Prednisolone ◽

Low Grade ◽

Evans Syndrome ◽

First Line ◽

Indirect Bilirubin ◽

First Line Therapy ◽

Line Therapy ◽

Immune Neutropenia ◽

Chronic Course

Evans syndrome is an uncommon haematological disorder characterised by autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia (ITP) and/or immune neutropenia. It may occur in all ethnic groups, all ages and has no sex predilection. The direct antiglobulin test (DAT) is almost invariably positive. This condition generally runs a chronic course and is characterised by frequent exacerbations and remissions. Corticosteroids and/or intravenous immunoglobulin (IVIG) are the most commonly used first line therapy. Here we report a case of a female who presented with severe shortness of breath, palpitation and low grade fever and on examination she was found severely pale and mildly icteric. Her CBC and PBF showed pancytopenia. Indirect bilirubin and LDH were raised and direct Coomb’s test was positive. She was labeled as a case of Evans syndrome and responded to oral prednisolone. On subsequent follow-up her haematological profiles remained normal. J Enam Med Col 2020; 10(2): 114-117

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A case report of Evans Syndrome

Faridpur Medical College Journal ◽

10.3329/fmcj.v12i2.34237 ◽

2017 ◽

Vol 12 (2) ◽

pp. 88-91

Author(s):

Muhammad Kamruzzaman ◽

Mohammad Iqbal Hossain ◽

MM Shahin Ul Islam ◽

Khan Mohammad Arif ◽

Md Towhid Alam ◽

...

Keyword(s):

Haemolytic Anaemia ◽

Immune Thrombocytopenia ◽

Oral Prednisolone ◽

Evans Syndrome ◽

Methyl Prednisolone ◽

Antiglobulin Test ◽

Immune Haemolytic Anaemia ◽

Immune Neutropenia ◽

Chronic Course

Evans syndrome is a haematological disorder characterized by the sequential or simultaneous development of direct antiglobulin test (DAT) positive autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia (ITP) and/or immune neutropenia in the absence of a known aetiology. No sex predilection is known. It may occur in all ethnic groups and all ages. This condition generally runs a chronic course and is characterized by frequent exacerbations and remissions. Here we report a case of a female who presented with both acute decompensated anaemia & thrombocytopenia. She received total 5 bags blood within 10 days for correction of anaemia but patients condition was deteriorating. CBC with PBF showed features consistent with immune haemolytic anaemia with thrombocytopenia, marked roulaeux formation and reticulocytosis. Direct Coomb's test (DAT) was positive and Indirect coomb's test was (IAT) negative. Her general condition was improved after injection methyl prednisolone for 3 days followed by oral prednisolone. Follow up CBC showed increased haemoglobin and platelet count without blood transfusion.Faridpur Med. Coll. J. Jul 2017;12(2): 88-91

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The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Blood ◽

10.1182/blood-2009-04-215368 ◽

2009 ◽

Vol 114 (15) ◽

pp. 3167-3172 ◽

Cited By ~ 122

Author(s):

Marc Michel ◽

Valérie Chanet ◽

Agnès Dechartres ◽

Anne-Sophie Morin ◽

Jean-Charles Piette ◽

...

Keyword(s):

Lymphoproliferative Disorders ◽

Severe Bleeding ◽

Evans Syndrome ◽

Related Risk ◽

Threatening Condition ◽

Life Threatening ◽

Underlying Disorder ◽

Immune Neutropenia ◽

Common Variable

Abstract Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia. To better describe the characteristics and outcome of ES in adults, a survey was initiated in 2005. The data from 68 patients (60% of them women) fulfilling strict inclusion criteria for ES are reported. The mean age at time of ITP and/or AIHA onset was 52 plus or minus 33 years, both cytopenias occurred simultaneously in 37 cases (54.5%). ES was considered as “primary” in 34 patients (50%) but was associated with an underlying disorder in half of the cases, including mainly systemic lupus, lymphoproliferative disorders, and common variable immunodeficiency. All patients were given corticosteroids, but 50 of them (73%) required at least one “second-line” treatment, including splenectomy(n = 19) and rituximab (n = 11). At time of analysis, after a mean follow-up of 4.8 years, only 22 patients (32%) were in remission off treatment; 16 (24%) had died. In elderly patients, the risk of cardiovascular manifestations related to AIHA seems to be higher than the ITP-related risk of severe bleeding. In conclusion, ES is a potentially life-threatening condition that may be associated with other underlying autoimmune or lymphoproliferative disorders.

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

Case Reports in Medicine ◽

10.1155/2021/8635585 ◽

2021 ◽

Vol 2021 ◽

pp. 1-4

Author(s):

Ivan Jose Ardila Gomez ◽

Pilar Pérez López ◽

Mónika Rocío Hernández Carreño ◽

Juan Camilo Barrios Torres

Keyword(s):

Immune System ◽

Rna Virus ◽

Dengue Infection ◽

Warning Signs ◽

Severe Dengue ◽

Evans Syndrome ◽

Economic Sectors ◽

Pathway Activation ◽

Activation Mechanisms ◽

Immune Neutropenia

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.

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The spectrum of Evans syndrome: a literature review

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20201958 ◽

2020 ◽

Vol 8 (5) ◽

pp. 1961

Author(s):

Hamza Maqsood ◽

Hassan Abdullah Shakeel ◽

Azouba Gulraiz ◽

Muhammad Dilawaer Khan

Keyword(s):

Autoimmune Disorder ◽

Underlying Disease ◽

Immune Disorders ◽

Front Line ◽

Evans Syndrome ◽

Sequential Development ◽

Line Therapy ◽

History Of ◽

Immune Neutropenia

Evans syndrome (ES) is an autoimmune disorder characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia in the absence of any underlying cause. Evans syndrome is a rare disorder although the exact frequency is unknown. No sex predilection is known and Evans syndrome has been described in all ethnic groups and at all ages. Classification of ES includes primary, with this being an exclusion diagnosis with no underlying condition, and secondary in the presence of an underlying disease. Clinical features are associated with anemia and thrombocytopenia including pallor, weakness, fatigue, jaundice, petechiae, ecchymosis, gingivorrhagia and epistaxis. First, a detailed history must be taken from the patient to determine the risk factors for developing ES then a family history of immune disorders along with a thorough physical examination. The management of Evans syndrome remains a challenge. Steroids with and without IVIG are recommended as front-line therapy. Red blood cell/platelet transfusion is indicated only in severe symptomatic patients due to the risk of exacerbations. Splenectomy may also be considered a second-line treatment.

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