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نویسندگان

  • Pedro Oliveira Raimundo
  • Susana Coelho
  • Alexandra Cabeleira
  • Luis Dias
  • Manuela Gonçalves
  • Julio Almeida
چکیده

The ovarian cystic teratoma is a rare cause of autoimmune haemolytic anaemia by warm antibodies, resistant to corticotherapy, with few case reports published in the medical literature. We present a case of a 45-year-old woman admitted to hospital due to general weakness. Laboratory studies revealed macrocytic anaemia, biochemical parameters of haemolysis and peripheral spherocytosis. The direct Coombs test was positive. Viral serologies, anti-nuclear antibodies, anti-double-stranded DNA antibodies and β2-microglobulin were negative. CT scan of the thorax, abdomen and pelvis showed a heterogeneous right anexial lesion. The patient was treated with corticotherapy without improvement of anaemia. Regression of extra-vascular haemolysis and normalisation of haemoglobin was obtained only after laparoscopic splenectomy and right ooforectomy, and the histopathology of the right anexial mass revealed a cystic teratoma. Previously published cases controlled the haemolysis by surgically removing the lesion associated with splenectomy. Background Immune haemolytic anaemia (IHA) is defined as an erythrocyte destruction by the combined action of complement factors and the reticuloendothelial system triggered by antibody bonding of erythrocyte antigens. The autoimmune form is a heterogeneous disease group characterised by endogenous production of antibodies against self-erythrocyte antigens. There is an estimated incidence of 1–3 cases per 100 per year and the causes include warm antibody autoimmune haemolytic anaemia (AIHA), cryoaglutinin syndrome, cold paroxystic haemoglobinuria and drug-induced AIHA. AIHA can be further classified as idiopathic or secondary with lymphoproliferative disease, autoimmune disorders and infectious diseases being the most common causes (table 1). Although rare, AIHA can be an early paraneoplasic phenomenon of non-lymphoid neoplasm-like dermoid cysts and ovarian teratoma, Kaposi sarcoma and carcinoid. Few cases of AIHA associated with ovarian neoplasm have been described, almost always associated to dermoid cysts, and treatment involves the surgical removal of the lesion. We report a case of AIHA associated with an ovarian teratoma with haemolytic regression after ooforectomy plus splenectomy. Case presentation A 45-year-old female patient attended our hospital because of shortness of breath, general weakness, fatigue and jaundice. The patient had been well until 3 weeks earlier when symptoms developed. She had a history of alcohol consumption of about 30 g/day and had no history of prior medication or recent drug exposure. Examination revealed only a discrete sclera icterus, pale skin and palpable hepatic tip (3 cm below right inferior costal border). Investigations 1 1 2 1 3 1

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تاریخ انتشار 2015