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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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Background: Evans syndrome is a rare autoimmune disorder characterized by simultaneous or sequential presence of a positive antiglobulin test, autoimmune haemolytic anemia (AIHA), and immune thrombocytopenia (ITP). It is characterised by frequent exacerbations and remissions within a chronic course. It was first described by Robert Evans in 1951. Incidence of AIHA is 1 per 75 - 80,000 and ITP is 5.5 /100000 per general adult population. Incidence of Evans syndrome is 1.8% to 10% of patients with ITP. Objective was to study the maternal and perinatal outcome of women with Evans syndrome (E).Methods: About 4 antenatal mothers were identified with Evans syndrome at St. Johns medical college and hospital, Bengaluru during the study period of 5 years from July 2013-July 2017. They were followed up during their antenatal, intra natal and postnatal period and outcomes were studied. All patients included in the study fulfilled the criteria for Evans syndrome.Results: There were 4 cases of Evans syndrome, with a total number of deliveries of 11859, during this 5 year study. Incidence was 0.09 per 1000 births. All patients presented with bleeding manifestations ranging from mucosal haemorrhage to subarachnoid haemorrhage (SAH) at the time of diagnosis. All patients were on treatment with either 1st or 2nd line of management with corticosteroids/ azathioprine. None had bleeding during pregnancy after the initiation of treatment. Patients had antenatal complications like preeclampsia 25%, IUGR 25%, oligohydraminos 50%, IUD 25%. 2 patients received platelet transfusions intrapartum. None had intrapartum or postpartum haemorrhage. There were no maternal and neonatal mortality.Conclusions: Evans syndrome in pregnancy is a rare condition and requires multi disciplinary approach involving specialists from obstetrics, neonatology, and hematology. Close maternal and fetal surveillance and management during pregnancy is essential to increase the possibility of a favourable pregnancy outcome in these women.
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Auto Immune Mixed Haemolytic Anaemia (AIHA) is defined as presence of both warm and cold antibodies against patient’s own red blood cells which is diagnosed by monospecific Direct anti-globulin test. Hereby we report a middle-aged women old women who was a known case of hypothyroidism on regular medication, presented with history of fatigability, exercise intolerance and exertional breathlessness of 1 month duration. The patient was subjected for preliminary investigations, which revealed severe anaemia with hemoglobin of 3.6 g% and an increased reticulocyte count of 12% with normal total leukocyte and platelet counts. Peripheral smear revealed anisopoikilocytosis, nucleated RBCs and schistocytes. Biochemical tests for haemolysis was evaluated which showed and elevated LDH levels (780U/L), and a reduced serum haptoglobulin levels. Liver Function test revealed a total bilirubin of 6.8mg/dl with indirect bilirubin of 5.4 mg/dl with normal liver enzymes. Baseline evaluation of Auto immune haemolytic anaemia with coombs test turned out to be positive. Patient was subjected for Mono specific DAT, Indirect Anti-globulin test (IAT) and antibody screening. Mono specific DAT showed both Anti IgG and anti C3 antibodies. IAT test was positive at 4⁰C and negative at 22⁰C and 39⁰C which confirmed that the AIHA was of mixed warm and cold type. On evaluation for connective tissue diseases, patient serum was reactive for ANA and ds-DNA and found to have Systemic Lupus Erythematosus which is a rarity and was responded to corticosteroids.
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Objetivo: presentar el caso de una gestante adolescente con síndrome de Evans, y hacer una revisión de la literatura respecto a su tratamiento y pronóstico durante el embarazo. Materiales y métodos: se presenta el caso de una paciente adolescente embarazada con síndrome de Evans, manejada en nuestra unidad, ubicada en un hospital de segundo nivel de referencia en Bogotá (Colombia); se describe la historia clínica, su diagnóstico, manejo y desenlace, y se realiza una revisión de la literatura con énfasis en su tratamiento y pronóstico. Se realizó una búsqueda de literatura utilizando las bases de datos Medline vía PubMed, Embase y la Biblioteca Cochrane a mayo de 2016. Las palabras clave utilizadas fueron "anemia hemolítica autoinmune", "trombocitopenia", "síndrome de Evans" y "embarazo", en español o inglés, sin límite por año de publicación. Resultados: se encontraron 79 publicaciones en la búsqueda en Medline y 61 en Embase. De estas, 13 estudios estaban directamente relacionados con el tema. Uno de los artículos corresponde a una revisión sistemática de la literatura y los demás a reportes de caso. Todos los reportes de caso encontrados están incluidos en la revisión sistemática. El síndrome de Evans se debe sospechar cuando se presenta trombocitopenia y hemólisis en la mujer gestante. La patología tiene un curso variable durante el embarazo y amerita un control estricto materno-fetal. Se dispone de alternativas que incluyen el uso de corticoides, gamaglobulina intravenosa y, en algunos casos, el manejo quirúrgico con esplenectomía. Conclusiones: el síndrome de Evans es una patología rara durante la gestación, se requieren más estudios respecto al tratamiento y pronóstico de la enfermedad que permitan guiar su manejo.
Objective: To present the case of a pregnant teenage girl with Evans Syndrome, and to conduct a review of the literature regarding treatment and prognosis during pregnancy. Materials and methods: Case presentation of a pregnant teenage girl with Evans Syndrome managed at our unit in a Level II referral hospital in Bogota, Colombia; description of the clinical history, diagnosis, management and outcome; and review of the literature focusing on treatment and prognosis. A search of the literature was conducted using the Medline database through PubMed, EMBASE and the Cochrane library up to May 2016. The key terms used were "autoimmune haemolytic anaemia", "thrombocytopenia", "Evans Syndrome" and "pregnancy", both in Spanish and English, with no restriction by year of publication. Results: Overall, 79 publications were found in Medline and 61 in EMBASE. Of these, 13 studies related directly to the topic, one was a systematic review of the literature, and the rest were case reports. All the case reports found are included in the systematic review. Evans Syndrome must be suspected when there is thrombocytopenia and haemolysis in the pregnant woman. The course of the disease varies during pregnancy and warrants close maternal and foetal follow-up. Treatment options are available, including steroids, intravenous gamma globulin and, in certain cases, surgical management with splenectomy. Conclusions: Evans Syndrome is a rare disease during pregnancy. Further studies are needed regarding the treatment and prognosis of the disease in order to guide treatment.