Primary Evans syndrome in an adult man

2021 ◽  
Vol 14 (12) ◽  
pp. e243051
Author(s):  
Archita Makharia ◽  
Manoj Lakhotia ◽  
Brateen Roy
Keyword(s):  
Haemolytic Anaemia ◽  
Immune Thrombocytopenia ◽  
Subsequent Development ◽  
Underlying Disease ◽  
Autoimmune Haemolytic Anaemia ◽  
Evans Syndrome ◽  
Haematological Disorders

Evans syndrome (ES) is a simultaneous or subsequent development of two haematological disorders, autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP). It can be primary (idiopathic) or secondary (associated with an underlying disease). Primary Evans is a diagnosis of exclusion and has a poorer prognosis than AIHA or ITP alone. We present a 55-year-old man who presented with weakness and lethargy and was diagnosed to be suffering from primary ES.

scholarly journals Epidemiology of Secondary Warm Autoimmune Haemolytic Anaemia—A Systematic Review and Meta-Analysis

2021 ◽  
Vol 10 (6) ◽  
pp. 1244
Author(s):  
Stinne Tranekær ◽  
Dennis Lund Hansen ◽  
Henrik Frederiksen
Keyword(s):  
Haemolytic Anaemia ◽  
Lupus Erythematosus ◽  
Meta Analysis ◽  
Underlying Disease ◽  
Age At Diagnosis ◽  
Autoimmune Haemolytic Anaemia ◽  
Sex Distribution ◽  
Number Of Patients ◽  
Study Results ◽  
Full Text Review

Background: Warm autoimmune haemolytic anaemia (wAIHA) is a haemolytic disorder, most commonly seen among adults and is classified as either primary or secondary to an underlying disease. We describe the age and sex distribution and the proportion of secondary wAIHA. Method: We retrieved 2635 published articles, screened abstracts and titles, and identified 27 articles eligible for full-text review. From these studies, we extracted data regarding number of patients, sex distribution, age at diagnosis, number of patients with secondary wAIHA, and whether the patients were diagnosed through local or referral centres. All data were weighted according to the number of included patients in each study. Results: 27 studies including a total of 4311 patients with wAIHA, of which 66% were females, were included. The median age at diagnosis was 68.7 years, however, wAIHA affected all ages. The mean proportion of secondary wAIHA was 49%, most frequently secondary to systemic lupus erythematosus. The proportions of secondary wAIHA reported from primary vs. referral centres were 35% vs. 59%, respectively. Conclusion: This review consolidates previously reported gender distribution. The higher proportion of secondary wAIHA in referral centres suggests that the most severely affected patients are disproportionally more frequent in such facilities.

CD5+ B lymphocytes secrete IL-10 rather than TGF-β1 which control the immune response in autoimmune haemolytic anaemia/Evans syndrome

Autoimmunity ◽  
2019 ◽  
Vol 52 (1) ◽  
pp. 12-20 ◽  
Author(s):  
Manjun Zhao ◽  
Ningning Duan ◽  
Yi Wang ◽  
Hongli Zhu ◽  
Hong Liu ◽  
...  
Keyword(s):  
Immune Response ◽  
B Lymphocytes ◽  
Haemolytic Anaemia ◽  
Autoimmune Haemolytic Anaemia ◽  
Evans Syndrome ◽  
Tgf Β1

scholarly journals A case report of Evans Syndrome

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

scholarly journals Evans Syndrome

1970 ◽  
Vol 11 (1) ◽  
pp. 78-82 ◽  
Author(s):  
Ahmedul Kabir ◽  
Jayanta Banik ◽  
Ratan Das Gupta ◽  
Robed Amin ◽  
AM Wasiq Faisal ◽  
...  
Keyword(s):  
Haemolytic Anaemia ◽  
Blood Cells ◽  
Clinical Evidence ◽  
Serum Bilirubin ◽  
White Blood Cells ◽  
Autoimmune Disorder ◽  
Blood Film ◽  
The Body ◽  
Autoimmune Haemolytic Anaemia ◽  
Evans Syndrome

Evans' syndrome is a rare autoimmune disorder in which the body makes antibodies that destroy the red blood cells, platelets and white blood cells. Patients are diagnosed with thrombocytopenia and Coombs' positive haemolytic anaemia and have no other known underlying aetiology. We report two cases of autoimmune haemolytic anaemia (AIHA) associated with immune thrombocytopenia which is known as Evans's syndrome. In these patient there were clinical evidence of haemolysis, serum bilirubin was elevated, blood film showed anaemia with trrombocytopenia but no spherocytosis. Immunosuppression was achieved with steroid. We would like to highlight that a negative Coomb's test is now often interpreted as absence of autoantibodies, but it is more or less preclusive for the diagnosis of AIHA. Keywords: Evan's Syndrome, autoimmune haemolytic anaemia DOI:10.3329/jom.v11i1.4280 J Medicine 2010: 11: 78-82

scholarly journals Evans Syndrome Complicated by Intratubular Hemoglobin Cast Nephropathy

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Iván González ◽  
Rehan Rais ◽  
Joseph P. Gaut ◽  
Louis P. Dehner
Keyword(s):  
Acute Kidney Injury ◽  
Renal Failure ◽  
Acute Renal Failure ◽  
Immune Thrombocytopenia ◽  
Kidney Injury ◽  
Subsequent Development ◽  
Autoimmune Disorder ◽  
Intravascular Hemolysis ◽  
Evans Syndrome ◽  
Cast Nephropathy

Evans syndrome (ES) is a rare autoimmune disorder whose exact pathophysiology is unknown. It is characterized by the simultaneous or subsequent development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Intravascular hemolysis, with hemoglobinemia, is known to produce acute kidney injury; however, the development of intratubular hemoglobin casts (hemoglobin cast nephropathy) in the setting of acute hemolysis is uncommon. Likewise, the association of ES and acute renal failure is equally uncommon. We present a case of a 7-year-old girl with ES who developed acute kidney injury in the setting of intravascular hemolysis and had widespread intratubular hemoglobin casts.

Autoimmune haemolytic anaemia: a review and report of four cases

2015 ◽  
Vol 156 (11) ◽  
pp. 449-456 ◽  
Author(s):  
Renáta Nyilas ◽  
Borbála Székely ◽  
László Váróczy ◽  
Zsófia Simon ◽  
Anita Árokszállási ◽  
...  
Keyword(s):  
Haemolytic Anaemia ◽  
Treatment Options ◽  
Immunosuppressive Agents ◽  
Underlying Disease ◽  
Autoimmune Haemolytic Anaemia ◽  
Line Treatment ◽  
First Line ◽  
Off Label ◽  
Severe Infections ◽  
First Line Treatment

Treatment of autoimmune haemolytic anaemia is still a challenge to clinicians. Even today it may be lethal. Half of the cases are secondary due to an underlying disease, and the others are primary or idiopathic cases. According to the specificity and type of autoantibodies there are warm and cold type forms of autoimmune haemolytic anaemia. The hallmark of the diagnosis is to detect the presence of haemolysis by clinical and laboratory signs and detect the underlying autoantibodies. Treatment of autoimmune haemolytic anaemia is still a challenge to clinicians. We still loose patients due to excessive haemolysis or severe infections caused by immunosuppression. First line treatment is corticosteroids. Other immunosuppressive agents like: cyclophosphamide, azathioprine, cyclosporine or the off label rituximab can be used in case of corticosteroid refractoriness. Splenectomy is a considerable option in selective cases. The authors discuss treatment options and highlight difficulties by presenting 4 cases. Orv. Hetil., 2015, 156(11), 449–456.

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