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      Impact of baseline fluorescent antinuclear antibody positivity on the clinical outcome of patients with primary autoimmune hemolytic anemia

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          Abstract

          Introduction

          Autoimmune haemolytic anaemia (AIHA) is an autoimmune disorder that can present in primary or secondary forms. The literature looking at impact of baseline fluorescent antinuclear antibody (FANA) positivity on outcomes of AIHA patients is infrequent.

          Objective

          To study the impact of baseline FANA positivity in patients with primary AIHA.

          Method

          A prospective cohort study involving 29 consecutive primary AIHA patients presenting to the Haematology department from 2013 to 2015 was analysed. After recording baseline investigations including fluorescent ANA, all patients were treated as per the standard therapeutic protocols. Clinical remission, disease free survival, relapse, mortality were compared between the FANA positive and FANA Negative AIHA groups.

          Results

          Baseline FANA positivity was found in 17 patients (58.62%). Both the groups were comparable in terms of age, sex, Hemoglobin, LDH at presentation, number of lines of treatment needed and duration of follow up. Evan's syndrome was seen in six of FANA positive patients which was statistically significant (0 v/s 6, p = 0.023). FANA positive patients had significantly higher rates of relapse per patient month follow up (1.22 v/s 3.57, p = 0.023) and lower rates of complete response (83.33% v/s 35.29%, p = 0.0118) and relapse free survival at five years. Morbidity and mortality were numerically higher in FANA positive patients.

          Conclusion

          Baseline FANA positivity among AIHA patients was found to be associated with lower complete response rates and higher relapse rates with possible higher rates of morbidity. Presence of FANA will give us prognostic value and help us in deciding the treatment options.

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          Most cited references17

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          Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients.

          The clinical outcome, response to treatment, and occurrence of acute complications were retrospectively investigated in 308 primary autoimmune hemolytic anemia (AIHA) cases and correlated with serological characteristics and severity of anemia at onset. Patients had been followed up for a median of 33 months (range 12-372); 60% were warm AIHA, 27% cold hemagglutinin disease, 8% mixed, and 5% atypical (mostly direct antiglobulin test negative). The latter 2 categories more frequently showed a severe onset (hemoglobin [Hb] levels ≤6 g/dL) along with reticulocytopenia. The majority of warm AIHA patients received first-line steroid therapy only, whereas patients with mixed and atypical forms were more frequently treated with 2 or more therapy lines, including splenectomy, immunosuppressants, and rituximab. The cumulative incidence of relapse was increased in more severe cases (hazard ratio 3.08; 95% confidence interval, 1.44-6.57 for Hb ≤6 g/dL; P < .001). Thrombotic events were associated with Hb levels ≤6 g/dL at onset, intravascular hemolysis, and previous splenectomy. Predictors of a fatal outcome were severe infections, particularly in splenectomized cases, acute renal failure, Evans syndrome, and multitreatment (4 or more lines). The identification of severe and potentially fatal AIHA in a largely heterogeneous disease requires particular experienced attention by clinicians.
            • Record: found
            • Abstract: not found
            • Article: not found

            Warm Autoimmune Hemolytic Anemia

              • Record: found
              • Abstract: not found
              • Article: not found

              How I treat autoimmune hemolytic anemia

                Author and article information

                Contributors
                Journal
                Hematol Transfus Cell Ther
                Hematol Transfus Cell Ther
                Hematology, Transfusion and Cell Therapy
                Sociedade Brasileira de Hematologia e Hemoterapia
                2531-1379
                2531-1387
                04 March 2022
                Apr-Jun 2023
                04 March 2022
                : 45
                : 2
                : 204-210
                Affiliations
                [a ]Assistant professor, Department of Microbiology, Gandhi Medical College, Bhopal, Madhya Pradesh, India
                [b ]Professor and Head, Department of Microbiology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
                [c ]Professor, Department of Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
                [d ]Associate Professor, Department of Medicine and Department of Adult Hematology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
                Author notes
                [* ]Corresponding author at: Department of Adult Hematology, Kasturba medical College Hospital, Mangalore 575001, India prashantha.b@ 123456manipal.edu
                Article
                S2531-1379(22)00033-5
                10.1016/j.htct.2022.01.013
                10244249
                35307304
                02c993c4-e7f4-4a7d-b155-999436cd0005
                © 2022 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier España, S.L.U.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 8 September 2021
                : 28 January 2022
                Categories
                Original Article

                anemia,autoimmune hemolytic anemia,evans syndrome,sle,antinuclear antibody

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