Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes

Abstract

and14 Manchester Royal Infirmary, Manchester, United Kingdom Key Points• In women presenting with an initial diagnosis of TTP during pregnancy, cTTP was more common than acquired TTP.• Active monitoring and management during pregnancy results in positive pregnancy outcomes.Pregnancy can precipitate thrombotic thrombocytopenic purpura (TTP). We present a prospective study of TTP cases from the United Kingdom Thrombotic Thrombocytopenic Purpura (UK TTP) Registry with clinical and laboratory data from the largest cohort of pregnancy-associated TTP and describe management through pregnancy, averting fetal loss and maternal complications. Thirty-five women presented with a first TTP episode during pregnancy: 23/47 with their first congenital TTP (cTTP) episode and 12/47 with acute acquired TTP in pregnancy. TTP presented primarily in the third trimester/ postpartum, but fetal loss was highest in the second trimester. Fetal loss occurred in 16/38 pregnancies before cTTP was diagnosed, but in none of the 15 subsequent managed pregnancies. Seventeen of 23 congenital cases had a missense mutation, C3178T, within exon 24 (R1060W). There were 8 novel mutations. In acquired TTP presentations, fetal loss occurred in 5/18 pregnancies and 2 terminations because of disease. We also present data on 12 women with a history of nonpregnancy-associated TTP: 18 subsequent pregnancies have been successfully managed, guided by ADAMTS13 levels. cTTP presents more frequently than acquired TTP during pregnancy and must be differentiated by ADAMTS13 analysis. Careful diagnosis, monitoring, and treatment in congenital and acquired TTP have assisted in excellent pregnancy outcomes. (Blood. 2014;124(2):211-219)

Current insights into thrombotic microangiopathies: Thrombotic thrombocytopenic purpura and pregnancy

“…There were 7 live births, 4 intrauterine fetal deaths and one pregnancy termination. All twelve women survived, 6 women had 8 subsequent pregnancies with one TTP relapse at 6 gestational weeks and one relapse postpartum as well as one IUFD (31). Furthermore Scully et al report on 18 pregnancies in 12 women having recovered from an acute acquired TTP not related to pregnancy.…”

Pregnancy Induced Microangiopathy, HELLP OR TTP!

“…The diagnosis is confirmed by documentation of ADAMTS13 mutation. 8 cTTP clinical signs may be mild or absent during childhood. History of easy bruising during childhood due to isolated thrombocytopenia can be the only symptom.…”

Egészségügyi szakmai irányelv – A thromboticus thrombocytopeniás purpura (TTP) és a haemolyticus uraemiás syndroma (HUS) kezeléséről

“…5%-a jelentkezik a terhesség ideje alatt. Ezek 46%-a a 30. terhességi hét után vagy post partum, 54%-a a 30. hét előtt (20-29. hét: 38%; a 20. hét előtt: 15%) kezdődik [98]. A terhesség alatti első TTP-epizódok kb.…”

“…A szülés optimális ideje a 36-38. terhességi hét. A betegeket ezt követően is ellenőrizni kell, mivel 20%-uk a továbbiakban is rendszeres plazmapótlásra szorul [98].…”

“…A magzatelhalás mindkét TTPtípusban a 2. trimesterben a leggyakoribb, ezért a placentában kialakuló microthrombosisok tehetők felelőssé. A terhesség elején kóros ADAMTS13-aktivitás a relapszus valószínűségét növeli [98], azonban a normális aktivitás sem garantálja a relapsuszmentes terhességet. Ismert inhibitoros TTP-s beteg esetében, az ADAMTS13-aktivitás sorozatos ellenőrzése (havonta) javasolt a terhesség alatt.…”