title: A case of severe ADAMTS13 deficiency presenting as thrombotic thrombocytopenic purpura in pregnancy.
abstract: BACKGROUND
  Thrombotic thrombocytopenic purpura is a rare life-threatening disorder characterized by thrombocytopenia and microangiopathic hemolytic anemia. It is caused by the absent or severe deficiency of the von Willebrand Factor-cleaving protease named ADAMTS13. Pregnancy is a well recognized factor precipitating the appearance of the disease both in women that had reduced levels of ADAMTS13 activity prior to gestation and in those with other inherited or acquired thrombophilic syndromes.
  
  
  METHODS
  We report a 25-year old woman with severe ADAMTS13 deficiency presented early in her 1st pregnancy and relapsed in two subsequent gestations. This presentation is uncommon for thrombotic thrombocytopenic purpura is associated with pregnancy (ADAMTS13 deficiency < 5%, without an inhibitor). In the first pregnancy she started with daily plasma exchange 1.5 x volume, corticosteroids and IV immunoglobulin and finally entered remission after 23 sessions and termination of pregnancy. In the second pregnancy she did not receive prophylactic treatment and relapsed in the 3rd trimester. Prophylactic treatment during the third pregnancy with plasma infusions proved also ineffective to prevent relapse.
  
  
  CONCLUSIONS
  Many issues regarding treatment and prevention of thrombotic thrombocytopenic purpura relapses in subsequent pregnancies are unclear. Proposed guidelines recommend that the same treatment should be performed on pregnant and non pregnant patients without modification of plasma replacement dose according to ADAMTS13 levels. In addition, many authors suggest that pregnant patients with history of thrombotic thrombocytopenic purpura and severe deficiency of ADAMTS13 levels should received prophylactic treatment for prevention of relapses in the subsequent pregnancies.
  
  
  CONCLUSIONS
  Severe ADAMTS 13 deficiency may present as thrombotic thrombocytopenic purpura of pregnancy. Pregnant women with thrombotic thrombocytopenic purpura and especially with severe deficiency of ADAMTS13 levels require specific consideration regarding treatment and prophylaxis in subsequent pregnancies.
authors.affiliation: Department of Obstetrics and Gynecology, University Hospital, Medical School of Patras, Greece. nikolaoumarinos@upatras.gr
authors.forename: Marinos
authors.initials: M
authors.lastname: Nikolaou
authors.forename: Marina
authors.initials: M
authors.lastname: Karakantza
authors.forename: George
authors.initials: G
authors.lastname: Adonakis
authors.forename: George
authors.initials: G
authors.lastname: Theodorou
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Zoumbos
authors.forename: George
authors.initials: G
authors.lastname: Decavalas
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000328:Adult; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D010951:Plasma Exchange; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D012008:Recurrence
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human