Maternal and perinatal outcome of Evan’s syndrome: a 5 years study in a tertiary care centre

Authors

  • Manjula S. K. Department of Obstetrics and Gynecology, St. John's Medical College Hospital, Bangalore, Karnataka, India
  • Jasmine Deva Arul Selvi T. Department of Obstetrics and Gynecology, St. John's Medical College Hospital, Bangalore, Karnataka, India
  • Vishnu Priya K. M. N. St.John's Medical College Hospital http://orcid.org/0000-0001-9430-8900

DOI:

https://doi.org/10.18203/2320-1770.ijrcog20192462

Keywords:

Autoimmune thrombocytopenia, Autoimmune haemolytic anaemia, Azathioprine, corticosteroids, Evans syndrome, IVIG

Abstract

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.

References

Kaushansky K, Williams WJ. Williams hematology. New York: McGraw-Hill Medical; 2010.

Mackay I, Rose NR. The autoimmune diseases. Netherlands: Elsevier.; 2006.

Sasidharan PK, Bindya M, Kumar KG. Hematological manifestations of SLE at initial presentation: Is it underestimated? ISRN Hematol. 2012;2012:961872.

Font J, Jimenez S, Cervera R, Garcia-Carrasco M, Ramos-Casals M, Campdelacreu J, et al. Splenectomy for refractory Evan’s syndrome associated with antiphospholipid antibodies: Report of two cases. Ann Rheum Dis. 2000;59(11):920-3.

Lavalle C, Hurtado R, Quezada JJ, Cabral A, Fraga A. Hemocytopenia as initial manifestation of systemic lupus erythematous. Prognostic significance. Clin Rheumatol. 1983;2(3):227-32.

Norton A, Roberts I, Management of Evans syndrome. Br J Haematol. 2006;132(2):125-37.

Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009;113(11):2386-93.

Buchanan GR, Adix L. Grading of hemorrhage in children with idiopathic thrombocytopenic purpura. J Pediatr. 2002;141(5):683-8.

Chaplin H, Cohen R, Bloomberg G, Kaplan HJ, Moore JA, Dorner I. Pregnancy and idiopathic autoimmune haemolytic anaemia: a prospective study during 6 months gestation and 3 months post-partum. Br J Haematol. 1973;24:219-29.

Lefkou E, Nelson-Piercy C, Hunt BJ Eur J Obstet Gynecol Reprod Biol. 2010 Mar; 149(1):10-7.

Letts HW, Kredenster B. Thrombocytopenia, haemolytic anemia, and two pregnancies. Report of a case. Am J Clin Pathol. 1968;49(4):481-6.

Clark AL. Clinical uses of intravenous immunoglobulin in pregnancy. Clin Obstet Gynecol. 1999;42:368-80.

Gill KK, Kelton JG. Management of idiopathic thrombocytopenic purpura in pregnancy. Semin Hematol. 2000;37:275-89.

Kaplan C. Immune thrombocytopenia in the foetus and the newborn: diagnosis and therapy. Transfus Clin Biol. 2001;8:311-4.

Boren T, Reyes C, Montenegro R, Raimer K. A case of Evan’s syndrome in pregnancy refractory to primary treatment options. J Matern Fetal Neonatal Med. 2007;20(11):843-5.

The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases -Marc Michel, Vale´rie Chanet, Agne`s Dechartres, Anne-Sophie Morin, Jean-Charles Piette - Blood First Edition paper, July 28;2009.

Mathew P, Chen G, Wang W. Evans syndrome: results of national survey. J Pediatr Hematol Onco. 1997;19(5):433-7.

Lewis, G (ed). The Confidential Enquiry into Maternal and Child Health. Saving Mothers' Lives: reviewing maternal deaths to make motherhood safer;2007.

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Published

2019-05-28

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Original Research Articles