EVANS SYNDROME: A CASE REPORT
Keywords:
Evans Syndrome, Spontaneous Bleeding, Antiphospholipid SyndromeAbstract
Evans Syndrome (ES) is a rare autoimmune disorder characterized by the presence of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP). The average patients were diagnosed at the age of 52 years with a female-to-male ratio of 3:2. A 43-year-old woman, with complaint of spontaneous bleeding of the gum ten hour prior to hospital. Pressure bandages have been applied but bleeding still occurs. There was no previous history of bleeding disorder or family history of bleeding disorder. There are ulcers that occur spontaneously on the left thigh and lower leg. The patient has a history of 5 miscarriages. The patient comes with stable vital signs. Physical examination shows anemic conjunctiva, active bleeding in the left gum, ulcers on the back of the left thigh and on the left lower leg. Laboratory findings showed hemoglobin 6.0, leukocytes 4770, platelets 79.000, reticulocytes 1.8%. BUN and electrolyte were normal. Peripheral blood morphology shows normochromic normocytic anemia with increased erythropoietic response and thrombocytopenia suggests a chronic process with bleeding. Immunoserological examination shows direct antiglobulin test (DAT) +1, immature platelet fraction (IPF) 7.9%. HIV test, anti-HCV and HBsag were non-reactive. The therapy given includes high-dose steroids, antibiotic, blood transfusions and symptomatic therapy. The patient was discharged after 6 days with clinical recovery. Laboratory findings of normochromic normocytic anemia with DAT +1 results suggest AIHA. Thrombocytopenia in a patient with elevated IPF suggest ITP. The presence of these two findings leads to the diagnosis of ES. A spontaneous ulcer can be a sign of blood vessel thrombosis that can occur in ES. History of recurrent miscarriage leads to secondary ES with antiphospholipid syndrome (APS). Futher blood test is needed to determine the cause of bleeding and antiphospholipid syndrome.
References
Jameson J, Fauci A, Kasper D, Hauser S, Longo D, Loscalzo J. Harrison’s Principles of Internal Medicine 20th ed. New York NY, McGraw Hill Educ. 2018;
Michel M, Chanet V, Dechartres A, Morin A-S, Piette J-C, Cirasino L, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. Oktober 2009;114(15):3167–72.
Hansen DL, Möller S, Andersen K, Gaist D, Frederiksen H. Evans syndrome in adults‐incidence, prevalence, and survival in a nationwide cohort. Am J Hematol. 2019;94(10):1081–90. [4] Audia S, Grienay N, Mounier M, Michel M, Bonnotte B. Evans’ Syndrome: From Diagnosis to Treatment. Vol. 9, Journal of Clinical Medicine. 2020.
Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, Gómez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018;9:171–84. [6] Giannakopoulos B; Krilis SA. The Pathogenesis of antiphospholipid syndrome. N Engl J Med. 2013;368(11):1033–44.
Antovic A; Sennstrom M; Bremme K; et al. Obstetric antiphospholipid syndrome. BMJ J. 2018;5:197–9.
Crowther M, Chan YLT, Garbett IK, Lim W, Vickers MA, Crowther MA. Evidence-based focused review of the treatment of idiopathic warm immune hemolytic anemia in adults. Blood. Oktober 2011;118(15):4036–40.
Neunert C, Terrell DR, Arnold DM, Buchanan G, Cines DB, Cooper N, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019;3(23):3829–66.
Chang DK, Yoo DH, Kim TH, Jun JB, Lee IH, Bae SC, et al. Induction of remission with intravenous immunoglobulin and cyclophosphamide in steroid-resistant Evans’ syndrome associated with dermatomyositis. Clin Rheumatol. 2001;20(1):63–6.
Wei Y, Ji X, Wang Y, Wang J, Yang E, Wang Z, et al. High-dose dexamethasone vs prednisone for treatment of adult immune thrombocytopenia: a prospective multicenter randomized trial. Blood, J Am Soc Hematol. 2016;127(3):296–302.
Mithoowani S, Gregory-Miller K, Goy J, Miller MC, Wang G, Noroozi N, et al. High-dose dexamethasone compared with prednisone for previously untreated primary immune thrombocytopenia: a systematic review and meta-analysis. Lancet Haematol. 2016;3(10):e489–96.
Godeau B, Chevret S, Varet B, Lefrère F, Zini J-M, Bassompierre F, et al. Intravenous immunoglobulin or highdose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: a randomised, multicentre trial. Lancet. 2002;359(9300):23–9.
Khellaf M, Michel M, Schaeffer A, Bierling P, Godeau B. Assessment of a therapeutic strategy for adults with severe autoimmune thrombocytopenic purpura based on a bleeding score rather than platelet count. Haematologica. 2005;90(6):829–32. [15] Buetens OW, Ness PM. Red blood cell transfusion in autoimmune hemolytic anemia. Curr Opin Hematol. 2003;10(6):429–33.
Goel R, Chopra S, Tobian AAR, Ness PM, Frank SM, Cushing M, et al. Platelet transfusion practices in immune thrombocytopenia related hospitalizations. Transfusion. 2019;59(1):169–76.
Provan D; Gribben JG. Molecular Hematology. 3 ed. Singapore: Wiley-Blackwell; 2015.
Spahr JE, Rodgers GM. Treatment of immune‐mediated thrombocytopenia purpura with concurrent intravenous immunoglobulin and platelet transfusion: a retrospective review of 40 patients. Am J Hematol. 2008;83(2):122–5.
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, et al. Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients. Blood, J Am Soc Hematol. 2014;124(19):2930–6.
Downloads
Published
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Licensing
Ninety Nine Publication publishes articles under the Creative Commons Attribution 4.0 International License (CC BY 4.0). This licensing allows for any use of the work, provided the original author(s) and source are credited, thereby facilitating the free exchange and use of research for the advancement of knowledge.
Detailed Licensing Terms
Attribution (BY): Users must give appropriate credit, provide a link to the license, and indicate if changes were made. Users may do so in any reasonable manner, but not in any way that suggests the licensor endorses them or their use.
No Additional Restrictions: Users may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.
