PB1541 - Atypical Hemolytic Uremic Syndrome Associated with Post-Partum Hemorrhage: A Case Series and Systematic Review of Individual Participants Data

Tuesday, June 27, 2023

18:30 – 19:30 ET

Room: Exhibition

Presenting Author(s)

Galia Spectre, MD PhD

Head of Thrombosis and Haemosasis Unit, Department of Hematology
Institute of Hematology, Rabin Medical Center, Petah Tikva
Petah Tikva, HaMerkaz, Israel

Co-Author(s)

SO

Sharon Orbach-Zinger

OBG anesthesiologist
Division of Anesthesia, Rabin Medical Center, Petah Tikva, Israel.
Petah Tikva, HaMerkaz, Israel
Avi Leader, MD (he/him/his)

Hematologist
Institute of Hematology, Rabin Medical Center, Petah Tikva
Petah Tikva, HaMerkaz, Israel
GS

Galia Stemer

Head department of Hematology
Hematology Institute, Galilee Medical Center
Nahariya, HaZafon, Israel
AW

Arnon Wiznitzer

Head of Helen Schider hsopital for women (retired)
Helen Schneider Hospital for Women, Rabin Medical Center, Petah Tikva, Israel
Petah Tikva, HaMerkaz, Israel
PS

Pierre Singer

Head of Intensive Care Unit, Rabin Medical Center (retired)
Intensive Care Unit, Rabin Medical Center, Petah Tikva, Israel
Petah Tikva, HaMerkaz, Israel
MD

Miriam Davidovits

Head of nephrology (retired)
Institute of Nephrology, Schneider Children’s Medical Center, Rabin Medical Center, Petah Tikva, Israel
Petah Tikva, HaMerkaz, Israel
MS

Michael Shapiro

Fellow in internal medicine
Department of Internal Medicine T, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
Tel Aviv, HaMerkaz, Israel
EH

Eva Hamyulya'k

resident in internal medicine
Department of Internal Medicine, Amsterdam UMC, Amsterdam, The Netherlands
Amsterdam, Noord-Holland, Netherlands
PR

Pia Raanani

Head division of Hematology
Institute of Hematology, Rabin Medical Center, Petah Tikva, Israel.
Petah Tikva, HaMerkaz, Israel
AG

Anna Gurevich-Shapiro

PhD Student Fellow in Internal medicine
Ido Amit Lab for Immunogenomics, Department of Systems Immunology, Weizmann Institute of Science, Rehovot, Israel
Rehovot, HaMerkaz, Israel

Background: Postpartum hemorrhage (PPH) has been recognized as a risk factor for pregnancy-associated atypical hemolytic uremic syndrome (aHUS) but has not been systematically studied.

Aims: To examine the role of PPH in precipitating aHUS, describe its characteristics management, and prognosis.

Methods: A systematic review of the English literature of case reports or series according to the PRISMA-IPD statement (Preferred Reporting Items for Systematic Reviews and Meta-Analyses of Individual Participant Data) and a case series from our institution. Search terms were ‘thrombotic microangiopathy’, ‘aHUS’ in combination with ‘pregnancy’ ‘post-partum’ and/or post-partum hemorrhage/haemorrhage. Exclusion criteria were other forms of TMA other than aHUS. (disseminated intravascular coagulation, HELLP syndrome, thrombotic thrombocytopenic purpura) Outcomes were clinical and laboratory characteristics of PPH-associated TMA/aHUS, treatment, and response.

Results: Apart from 3 patients from our institution, the search yielded 27 studies describing 42 women with PPH-associated TMA/aHUS. (Figure 1) Most women presented at term(58%) and had pregnancy complications(22/45,49%), mainly pre-eclampsia(15/45,33%) or fetal demise(9/45,20%). Cesarean section, particularly emergent, was the most common mode of delivery.
Hematological and renal abnormalities usually appeared within the first 24 hours post-delivery. The mean platelet count was 49x109/L (range 9-102), the mean maximal LDH was 2937 IU (range 593-8390) and 32 women (76%) underwent dialysis. While renal function normalized in all 18 women treated with C5-inhibitors (with or without plasma exchange) and in 7/10(70%) women treated with plasma exchange alone, it normalized only in 3/17(18%) of patients treated with supportive care only. (Figure 2), p< 0.001 for C5-inhibitors ± plasma exchange vs supportive care. The addition of C5 inhibitors to plasma exchange demonstrated superior outcomes (p=0.01).

Conclusion(s): aHUS is a rare and unique complication following PPH. It mainly occurred in women with pre-eclampsia and/or following cesarean section. Patients treated with C5 inhibitors and/or plasma exchange had a better renal prognosis compared with patients who received supportive treatment alone.