Evans' syndrome- haemolytic anaemia with thrombocytopenia - a rare autoimmune disorder

Momin, Majed Abdul Basit; Aluri, Anamika; Reddy, S. Jithender; Pasupala, Nanda Kishore

doi:10.15380/2277-5706.jcsr.17.08.004

Cited by 3 publications

(3 citation statements)

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“…The highest prevalence of warm and mixed AIHA, and Evan's Syndrome was among Malay patients, while cold AIHA was the highest among Chinese patients. It has been reported there is no racial predilection for AIHA and Evan's Syndrome (Gehrs & Friedberg 2002;Momin et al 2017). Currently, we do not have an explanation for the higher prevalence of cold AIHA among Chinese patients and further research is needed to explore the association.…”

Section: Discussionmentioning

confidence: 83%

Autoimmune Haemolytic Anaemia: A cross sectional study in a Tertiary Haematological Centre

SULAIMAN¹,

SINGH

HISHAM³

et al. 2022

JSKM

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Autoimmune haemolytic anaemia (AIHA) is a group of disorders wherein autoantibody causes decompensated acquired haemolysis. There has been no epidemiological study of autoimmune haemolytic anaemia (AIHA) in Malaysia. This study retrospectively analysed the epidemiology of AIHA including Evan’s Syndrome in a Tertiary Haematology Centre in Malaysia. Patients diagnosed with AIHA and Evan’s Syndrome at 18 years old and above between 1 January 1994 to 1 October 2020 at the out-patient Haematology Clinic of Hospital Raja Permaisuri Bainun, Ipoh were selected. Patients’ information was retrieved from the outpatient clinic records. A total of 71 patients were included of which predominantly female. The mean age for both genders were comparable. Ethnic stratification revealed AIHA was higher in Malays followed by Chinese and Indian. Warm AIHA was most prevalent at 40.8%, compared to cold AIHA and Evan’s Syndrome (both 23.9%), and mixed AIHA (11.3%). Primary was more common than secondary AIHA followed by Evan’s Syndrome. Approximately half of the secondary AIHA and secondary Evan’s Syndrome were due to SLE. Overall, 67.6% of patients received corticosteroid only and 28.2% combined with immunosuppressant. Individuals at higher age and females have higher risk of developing AIHA and Evan’s Syndrome. The highest prevalence was seen among the Malay ethnic. Primary warm AIHA is the most common type and majority of Evan’s syndrome are secondary to autoimmune diseases.

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Section: Discussionmentioning

confidence: 83%

Autoimmune Haemolytic Anaemia: A cross sectional study in a Tertiary Haematological Centre

SULAIMAN¹,

SINGH

HISHAM³

et al. 2022

JSKM

View full text Add to dashboard Cite

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“…Unlike immune-mediated hemolytic anemia (IMHA) which targets red blood cells, ITP manifests with the body's immune system excessively targeting platelets, leading to their depletion at a pace surpassing their production in the bone marrow. Evan's syndrome is a rare autoimmune disorder that poses significant diagnostic and therapeutic challenges in veterinary medicine [10]. Researchers claim that 30% of animals with IMTP also develop IMHA at the same time [11].…”

Section: Resultsmentioning

confidence: 99%

Evan’s Syndrome Hemolytic Anemia with Thrombocytopenia in a Labrador Dog: A Case Report

Chandrakant,

Shukla,

Chaudhary

et al. 2024

UPJOZ

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A seven-year-old Labrador retriever intact male dog was presented to the Referral Veterinary Polyclinic, Indian Veterinary Research Institute, Izatanagar with a history of inappetance, melena, and petechial hemorrhages on the ventral abdomen and treated by a local veterinarian for a prolonged period of time. Clinical examination showed pale mucous membranes, peripheral lymphadenopathy, and mild hepatomegaly. A Complete blood count revealed anemia, leukopenia, and thrombocytopenia and serum biochemistry showed hypoalbuminemia and hypoproteinemia. The dog was suspected of having a haemoprotozoan infection, and a thin peripheral blood smear was submitted to the Division of Parasitology, Indian Veterinary Research Institute, Izatanagar, Bareilly, for examination. A blood smear was screened for haemoprotozoan examination using Giemsa’s stain, which showed positivity for Babesia vogeli. An abdominal ultrasound confirmed hepatic enlargement. The blood clotting profile reveals an increase in activated partial thromboplastin time and prothrombin time. The dog's blood sample tested negative for Babesia vogeli, B. gibsoni, E. canis, and H. canis by multiplex PCR; this could be because of prior initiation of antibiotics. A periheral blood smear showed elevated spherocytes count and saline agglutination test was positive. Based on history, clinical examinations, laboratory findings and cytological examinations the case was tentatively diagnosed as chronic case of tick fever with concomitant Evans syndrome. A blood transfusion was performed and around 300 ml of whole blood was infused. Treatment was initiated with triple antibiotic therapy and an immunosuppressive dose of steroids, but after 4 weeks, the dog succumbed to the condition. Further research is required to optimize treatment regimens for dogs with Evan's syndrome, as the prognosis is uncertain.

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“…Evans syndrome is an autoimmune disorder described by Robert Evans in 1951 specifying that a link exists between primary thrombocytopenic purpura and acquired haemolytic anaemia. 1 It is a rare autoimmune disorder characterised by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Approximately, Evans syndrome represent 5-10% of warm autoimmune hemolytic anemia cases and 2-5% of ITP cases.…”

Section: Introductionmentioning

confidence: 99%

A case of Evans syndrome

2022

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Evans syndrome is an autoimmune disorder described by Robert Evans in 1951 specifying that a link exists between primary thrombocytopenic purpura and acquired haemolytic anaemia. It is a rare autoimmune disorder characterised by the simultaneous or sequential development of autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Approximately, Evans syndrome represent 5-10% of warm autoimmune haemolytic anaemia cases and 2-5% of ITP cases. It is basically a diagnosis of exclusion. We present a 50-year-old female presented with anaemia, jaundice and petechia. Patient indirect bilirubin was raised, Coombs test was positive, lactate dehydrogenase and reticulocyte count were raised. Based on the Coombs positive haemolytic anaemia and thrombocytopenia patient was diagnosed with Evans syndrome. ANA profile was positive for SS-A suggestive of underlying autoimmune disorder. Evans syndrome is difficult to diagnose and treat. Typically, corticosteroids and other immunosuppressive medications are used to manage the syndrome.

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Evans' syndrome- haemolytic anaemia with thrombocytopenia - a rare autoimmune disorder

Cited by 3 publications

References 0 publications

Autoimmune Haemolytic Anaemia: A cross sectional study in a Tertiary Haematological Centre

Autoimmune Haemolytic Anaemia: A cross sectional study in a Tertiary Haematological Centre

Evan’s Syndrome Hemolytic Anemia with Thrombocytopenia in a Labrador Dog: A Case Report

A case of Evans syndrome

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