Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes
| Autor: | Mary Underwood, Patrick O Brien, Fionnuala Ní Áinle, William Lester, Michael J. Nash, Marie Scully, Siobhan McGuckin, Katherine Langley, Henry G. Watson, Rosemary J. Scott, Mari Thomas, Rhona Maclean, Desmond Creagh, Raymond Camilleri, G. Evans, Ashok Roy, Rachel Rayment, Amanda Clark, Vickie McDonald |
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| Rok vydání: | 2014 |
| Předmět: |
Pediatrics
medicine.medical_specialty Placenta DNA Mutational Analysis Immunology Thrombotic thrombocytopenic purpura ADAMTS13 Protein Congenital Thrombotic Thrombocytopenic Purpura Biochemistry Cohort Studies Pregnancy hemic and lymphatic diseases medicine Humans heterocyclic compounds Prospective cohort study Upshaw–Schulman syndrome neoplasms Purpura Thrombotic Thrombocytopenic business.industry Pregnancy Complications Hematologic Pregnancy Outcome Cell Biology Hematology respiratory system medicine.disease ADAMTS13 ADAM Proteins Aborted Fetus Cohort Female business therapeutics Cohort study |
| Zdroj: | Blood. 124:211-219 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood-2014-02-553131 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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