Vol.:(0123456789) 1 3 Journal of Thrombosis and Thrombolysis https://doi.org/10.1007/s11239-020-02133-4 Pregnancy and non‑pregnancy related immune thrombotic thrombocytopenic purpura in women of reproductive age Amihai Rottenstreich 1  · Shira Dor 2  · Anat Keren‑Politansky 3  · Galit Sarig 3  · Yona Nadir 3  · Martin Ellis 4,5  · Galia Spectre 5,6  · Ilya Kirgner 5,7  · Oleg Pikovsky 8  · Ariela Arad 9  · Eldad J. Dann 10  · Yosef Kalish 9 © Springer Science+Business Media, LLC, part of Springer Nature 2020 Abstract Pregnancy is a precipitating factor for immune thrombotic thrombocytopenic purpura (iTTP). We compared the clinical course and outcomes of iTTP in women of reproductive age, between those with pregnancy- and non-pregnancy-related iTTP. A review of all reproductive-aged women diagnosed with iTTP during 2010–2019 in seven university hospitals in Israel. Of 42 cases of iTTP, 12 (28.6%) were pregnancy-related. At presentation, the laboratory profles did not difer signifcantly between those with pregnancy- and non-pregnancy-related iTTP, including hemoglobin (median 8.4 vs 8.0 g/dL), platelet count (12.5 vs. 11.5 X 10 9 /L); and levels of bilirubin (1.23 vs. 1.82 mg/dL), lactate dehydrogenase (1615 vs. 1701 U/L), creatinine (0.61 vs. 0.79 mg/dL) and anti-ADAMTS13 antibodies titer (75 vs. 82 U/mL). The proportions of women with renal, neurologic, or hepatic involvement were similar between the groups. Cardiac involvement was more common among those with pregnancy-related disease (25.0% vs. 3.3%, P = 0.06). The median number of courses of plasma-exchange therapy was 11 for both groups. All the women were treated with parenteral corticosteroids and the rate of adjunctive treatments did not difer between the groups (P = 0.30). Four women (one-third) with pregnancy-related disease had preeclampsia. Two women (16.7%) with pregnancy-related iTTP died during the acute episode (P = 0.07); no deaths were observed in the non- pregnancy-related group. Among reproductive-aged women with iTTP, most clinical and laboratory profles were similar between those with pregnancy- and non-pregnancy-related disease. However, the higher rates of cardiac involvement and mortality among women with pregnancy-related iTTP highlight its challenging management. Keywords Immune · Outcomes · Pregnancy · Reproductive-age · Thrombotic thrombocytopenic purpura Abbreviations GA Gestational age iTTP Immune thrombotic thrombocytopenia purpura LDH Lactate dehydrogenase Amihai Rottenstreich and Shira Dor have contributed equally to this work. Electronic supplementary material The online version of this article (https://doi.org/10.1007/s11239-020-02133-4) contains supplementary material, which is available to authorized users. * Yosef Kalish ykalish@gmail.com 1 Department of Obstetrics and Gynecology, Hadassah- Hebrew University Medical Center, 91120 Jerusalem, Israel 2 Faculty of Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel 3 Thrombosis and Hemostasis Unit, The Bruce Rappaport Faculty of Medicine, Technion, Rambam Health Care Campus, Haifa, Israel 4 Institute of Hematology, Meir Medical Center, Kfar Saba, Israel 5 Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel 6 Coagulation Unit, Institute of Hematology, Beilinson Hospital, Rabin Medical Center, Petah Tikva, Israel 7 Division of Hematology, Sourasky Medical Center, Tel Aviv, Israel 8 Transfusion Medicine Institute, Hematology Institute, Soroka University Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel 9 Hematology Department, Hadassah-Hebrew University Medical Center, POB 12000, Jerusalem, Israel 10 Blood Bank and Apheresis Unit, The Bruce Rappaport Faculty of Medicine, Technion, Rambam Health Care Campus, Haifa, Israel