Recombinant ADAMTS13 for Hereditary Thrombotic Thrombocytopenic Purpura.

N Engl J Med

From Zentrum für Perioperative Thrombose und Hämostase (L.M.A.), Nephrology and Dialysis (A.S., J.H.-S.), Obstetrics and Gynecology (A.K.), Emergency Center (A.L.), and Institute for Anesthesiology and Intensive Care (E.L., M.T.G.), Hirslanden Clinic (L.M.A.), the Institute of Pathology Enge and Muensterlingen (S.H., M.T.), and the Medical Faculty, University of Zurich (L.M.A., A.S., A.K., A.L., M.T.G., M.T.), Zurich, and the Department of Hematology and Central Hematology Laboratory, Bern University Hospital, University of Bern, Bern (J.A.K.H.) - all in Switzerland.

Published: December 2022

AI Article Synopsis

  • A 27-year-old woman with a history of severe pregnancy issues and blood clots was diagnosed with hereditary thrombotic thrombocytopenic purpura (TTP) due to a lack of the ADAMTS13 protein during her second pregnancy.
  • She started receiving weekly injections of recombinant ADAMTS13 when her condition worsened and the fetus was at risk, which helped normalize her platelet count and stabilize fetal growth.
  • At 37 weeks, she successfully delivered a small but healthy boy via cesarean section, and both continue to do well with ongoing treatment every two weeks.

Article Abstract

A 27-year-old patient with a history of severe obstetrical complications and arterial thrombosis received a diagnosis of hereditary thrombotic thrombocytopenic purpura (TTP) due to severe ADAMTS13 deficiency when she presented with an acute episode in the 30th week of her second pregnancy. When the acute episode of hereditary TTP became plasma-refractory and fetal death was imminent, weekly injections of recombinant ADAMTS13 at a dose of 40 U per kilogram of body weight were initiated. The patient's platelet count normalized, and the growth of the fetus stabilized. At 37 weeks 1 day of gestation, a small-for-gestational-age boy was delivered by cesarean section. At the time of this report, the patient and her son were well, and she continued to receive injections of recombinant ADAMTS13 every 2 weeks. (Funded by the Swiss National Science Foundation.).

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Source
http://dx.doi.org/10.1056/NEJMoa2211113DOI Listing

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