Evans syndrome and pregnancy: a case report

Abstract

Evans syndrome is characterised by simultaneous or sequential development of autoimmune thrombocytopenia and autoimmune haemolytic anaemia in the absence of a known underlying cause.1,2 Autoantibodies against erythrocytes, platelets and neutrophils have been shown in Evans syndrome. Both the occurrence of isolated episodes of thrombocytopenia and haemolytic anaemia and the results of in vitro studies have suggested the roles of noncrossreacting autoantibodies targeted at different antigenic determinants on red cells and platelets. Both humoral and cellular immune response abnormalities have been shown in Evans syndrome. 1There have been a few case reports of Evans syndrome during pregnancy. 3,4 We present a case of Evans syndrome during pregnancy, complicated by mild pre-eclampsia and fetal death in utero. Case reportA 19 year old primigravid woman presented initially for antenatal care at 13 weeks of pregnancy on April 9, 2002. Evans syndrome had been diagnosed last year, and she was taking prednisolone 20 mg daily. She presented with anaemia, splenomegaly and thrombocytopenia at the first time of diagnosis. Laboratory investigations at that time revealed thrombocytopenia, positive direct and indirect Coombs test and positive anti-IgG. Antinuclear, anticardiolipin and antiphospholipid antibodies were all negative. The prednisolone dose was increased to 60 mg daily and was gradually reduced to 20 mg daily. On physical examination at the first antenatal visit, her blood pressure was 100/60 mmHg, pulse rate was 80/min and respiratory rate was 20/min. Her height and weight were 162 cm and 65 kg, respectively. The uterine size was consistent with 18 weeks of pregnancy. Initial blood tests included a haematocrit of 31.8%, a white blood cell count of 12,500 cells/mm 3 with 81.9% neutrophils and a platelet count of 68,000/mm 3 . The prothrombin time and partial thromboplastin time were normal. A VDRL test was positive 1:4, and a TPHA test was reactive. A HBsAg test was negative as was anti-HIV testing. Ultrasonography revealed a single viable fetus compatible with 18 weeks of pregnancy. The dose of prednisolone (20 mg daily) was continued. She was also prescribed intramuscular benzathine penicillin 2.4 megaunits weekly for three weeks for treatment of syphilis in pregnancy (treated as latent syphilis of unknown duration) as recommended from the Centers for Disease Control 5 after the platelets increased. The pregnancy was unremarkable until 31 weeks of gestation. She was then admitted into the hospital because of mild pre-eclampsia. Specialists in perinatology, haematology and infectious medicine were consulted to look after her. Her blood pressure was 140/90 mmHg and she had 1+ proteinuria. The haematocrit level was 35.6% and the platelet count was 62,000/mm 3 on admission. Ultrasonography demonstrated a normal fetus with an estimated fetal weight of 1475 g, approximate for 31 weeks of gestation. The dose of prednisolone was increased to 60 mg daily in view of the fall in the number of platelets. The platelet count re...

Síndrome de Evans y embarazo en la adolescencia: reporte de un caso y revisión de la literatura

“…Tres pacientes no presentaron síntomas al mo mento del diagnóstico y este se realizó por el antecedente (9) o por los hallazgos paraclínicos (anemia, trombocitopenia) en dos casos (14,15); cinco pacientes presentaron sangrado clínico que incluyó epistaxis, sangrado gastrointestinal o hematuria (10)(11)(12)(13)16). Una paciente debutó con ictericia en el primer trimestre de la gestación (15), y de las pacientes reportadas por Lefkou (6), una presentó síndrome febril y pancitopenia y otra preeclampsia.…”

“…Cuatro de estos nacimientos fueron vaginales (9,13,15,16) y uno por cesárea de emergencia (12); no se presentaron desenlaces fatales para los fetos-recién nacidos en esos casos. Los demás fueron nacimientos pretér-mino (6,10,11,14,15), tres de estos fueron por cesárea de emergencia (6,14).…”

“…Respecto a complicaciones específicas, en tres pacientes se reportó el diagnóstico de trastornos hipertensivos asociados al embarazo, que incluyó dos síndromes HELLP (6, 12) y una preeclampsia sin criterios de severidad (11). En ninguno de los casos reportados parece haberse presentado neutropenia al momento del diagnóstico, que sí es común en niños y adultos (2,4).…”

“…En cuanto al pronóstico perinatal se reportaron dos muertes fetales (11,15). En ambos casos se demostró paso transplacentario de anticuerpos con hemólisis masiva y muerte fetal in utero, por lo que consideramos que podría estar indicada la búsque-da activa de anemia fetal en este tipo de pacientes como en otros de riesgo.…”

A case of Evan's syndrome in pregnancy refractory to primary treatment options

“…Using the MEDLINE search engine, only three previous articles cite Evan's syndrome during pregnancy [1][2][3]. One describes anesthetic complications with performing a second trimester splenectomy in a patient with Evan's syndrome.…”