HELLP syndrome is a life-threatening condition that may potentially cause complications during pregnancy. If not diagnosed and treated quickly, HELLP syndrome may lead to serious complications both for the mother and the baby. The aim of this study was to determin the effectiveness of therapeutic plasma exchange (TPE) for treatment of Class-I HELLP syndrome.

Laboratory results from 47 patients with Class-I HELLP syndrome patients who underwent TPE between 2011 and 2020 were recorded before and after the procedure. A central venous catheter was inserted, and TPE was performed in patients who had not responded to delivery, steroid, and supportive therapy (blood products, anti-hypertensive therapy, intravenous fluid administration, and antibiotics) within 24 hours after the diagnosis of Class I HELLP syndrome according to the Mississippi Criteria.

The average age of patients was 33 ± 4.7 years (range; 21-39 years). A mean of 5 (range; 4 to 6) TPE sessions were performed. There was a statistically significant decrease in total bilirubin, lactic dehydrogenase, aspartate aminotransferase, and alanine aminotransferase levels in all patients, whereas a significant increase in platelet count was observed (p < 0.05). Furthermore, clinical and laboratory improvement was achieved.

In all patients with HELLP syndrome, a dramatically clinical and laboratory improvement occurred after TPE. Our study suggests that postpartum use of TPE within 24 hours is an efficient treatment option for Class-I HELLP syndrome.

To evaluate effectiveness and safety of therapeutic plasma exchange (TPE) and dextran-sulfate plasma adsorption (DSA) for extracorporeal removal of soluble Fms-like tyrosine kinase-1 (sFlt-1) as part of expectant management of preeclampsia at extremely preterm gestational age.

Retrospective case series of six patients with preeclampsia at <28 weeks of gestation, treated with DSA or TPE. Laboratory results, clinical characteristics and neonatal outcomes were collected from charts and National Perinatal Information System.

Fetal growth restriction (FGR) was diagnosed in all cases. Pregnancy was prolonged for a median of 14 (range 5-74) days from admission and 10 (3-73) days from first apheresis. A mixed effects model showed a decrease in sFlt-1 and sFlt-1/PlGF ratio during DSA/TPE (significant effect of time [before/after]), which was comparable between DSA and TPE (no effect of procedure type). Median absolute reduction in sFlt-1 was 42% (inter-quartile range [IQR] 13%-57%) during DSA and 34% (16%-40%) during TPE; for sFlt-1/PlGF ratio it was 29% (22%-36%) and 38% (29%-42%), respectively. All procedures were well tolerated by fetuses. Anaphylactoid reaction, often with angioedema, occurred in 4/6 patients undergoing DSA and was attributed to bradykinin activation. One patient developed wound hematoma after cesarean section, possibly attributed to depletion coagulopathy.

As potential novel treatment of early preeclampsia, a non-selective and widely available TPE was comparable to DSA regarding sFlt-1 reduction but was associated with fewer side-effects. Both seem to allow maternal stabilization and pregnancy prolongation even when early preeclampsia is complicated by FGR.

Proteinuria is a frequently detected symptom, found in 20% of pregnancies. A common reason for proteinuria in pregnancy is preeclampsia. To diagnose preeclampsia clinically and to get new insights into the pathophysiology of the disease it is at first essential to be familiar with conditions in normal pregnancy. Animal models and biomarkers can help to learn more about disease conditions and to find new treatment strategies. In this article we review the changes in kidney function during normal pregnancy and the differential diagnosis of proteinuria in pregnancy. We summarize different pathophysiological theories of preeclampsia with a special focus on the renal facets of the disease. We describe the current animal models and give a broad overview of different biomarkers that were reported to predict preeclampsia or have a prognostic value in preeclampsia cases. We end with a summary of treatment options for preeclampsia related symptoms including the use of plasmapheresis as a rescue therapy for so far refractory preeclampsia. Most of these novel biomarkers for preeclampsia are not yet implemented in clinical use. Therefore, we recommend using proteinuria (measured by UPC ratio) as a screening parameter for preeclampsia. Delivery is the only curative treatment for preeclampsia. In early preeclampsia the primary therapy goal is to prolong pregnancy until a state were the child has an acceptable chance of survival after delivery.

This review summarizes the clinical evidence and practical details for the use of plasmapheresis and other apheresis modalities for each indication in nephrology. Updated information on the molecular biology and immunology of each renal disease is discussed in relation to the rationale for apheresis therapy and its place amid other available treatments. Autoantibody-mediated diseases, such as anti-GBM (anti-glomerular basement membrane) glomerulonephritis (GN), ANCA (antineutrophil cytoplasmic antibody)-related GN and the antibody-mediated type of TTP (thrombotic thrombocytopenic purpura), and alloantibody-mediated diseases such as kidney transplant sensitization and humoral rejection, can be treated by various plasmapheresis methods. These include standard plasmapheresis with a replacement volume, or plasmapheresis with online plasma purification using adsorption columns or secondary filtration. However, it should be noted that the pathogenic molecules implicated in FSGS (focal segmental glomerulosclerosis), myeloma cast nephropathy, and perhaps other diseases are too small to be removed by most online purification methods. A great majority of controlled trials and series on which evidence-based treatment recommendations are made were performed using centrifugal plasmapheresis; it is presumed that membrane-separation plasmapheresis is equally efficacious. For some rarer diseases, such as MPGN (membranoproliferative GN) type 2 with factor H abnormalities or C3Nef (C3 nephritic factor) autoantibodies, there are only a few case reports, but enough scientific understanding to warrant a trial of plasmapheresis in severe cases. Photopheresis, which is effective for cell-mediated rejection in heart and lung transplantation, has not yet found a place in the routine treatment of kidney transplant rejection.

Thrombotic thrombocytopenic purpura (TTP) commonly affects women of childbearing age. Although cases of TTP in pregnancy have been reported, the pathology of TTP in the placenta has not been described. In other organs, TTP has a distinctive pathology of arteriolar aneurysms with segmental intramural hyaline deposits. Published many decades ago, these classic papers antedate modern computerized literature searches, so this information has not been included in many modern reviews on TTP. We report 2 cases of TTP in pregnancy, both leading to fetal loss in the 2nd trimester. We noticed a distinctive pathology of TTP in the spiral arteries on the maternal surface of the placenta, particularly the "snowman sign," which differs somewhat from TTP in other organs due to the fact that TTP is superimposed on trophoblast-induced changes in these spiral arteries. In one of our cases, where the clinical diagnosis of TTP was difficult, the distinctive placental findings helped reinforce the clinical decision to perform potentially life-saving plasmapheresis.

Current reimbursement policy of health insurance for therapeutic plasmapheresis requires proof of efficacy using the concept of evidence-based medicine. The aim of this paper is to review the outcome of plasmapheresis used to treat thrombotic microangiopathy (TMA)-associated syndromes in the last decade to provide scientific evidence to back up reimbursement applications. The strength of evidence of each reviewed study was assessed using the five levels of evidence criteria as defined by the American Society of Hematology in 1996 for assessment of the treatment of immune thrombocytopenia. The level Experimental indication was added for situations where only case reports or small series supported by pathophysiological reasoning are available. The definitions of evidence used in this paper are as follows: Level I, randomized clinical trial with low rates of error (p < 0.01); Level II, randomized clinical trial with high rates of error (p < 0.05); Level III, nonrandomized studies with concurrent control group; Level IV, nonrandomized studies with historical control group; Level V, case series without a control group or expert opinion; and Experimental, case reports and pathophysiological reasoning. The results of this analysis based on the published data is summarized as follows: The indication of plasmapheresis is assigned to Level IV evidence for thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS); cancer/chemotherapy-associated TTP/HUS is assigned to Level V evidence; and TTP/HUS refractory to standard plasma exchange and post-bone marrow transplantation TTP/HUS are assigned to Experimental indication. For both subsets, protein A immunoadsorption is reportedly successful. The other TMA-associated syndromes, hemolysis elevated liver enzymes low platelets and HUS in early childhood, are no indication of plasmapheresis. Two randomized clinical trials were performed in order to demonstrate the superiority of plasma exchange/fresh frozen plasma (PEX/FFP) over plasma transfusion in the management of TTP/HUS. The results prove the greater clinical success of the latter type of plasma administration. Standard PEX/FFP has reduced the mortality of TTP/HUS from 94.5% to 13%.

The complicated preeclamptic patient represents a challenge for the clinician faced with her antepartum or postpartum care. The most serious sequelae of preeclampsia account for a significant portion of maternal morbidity and mortality. Severe preeclampsia also results in an appreciable portion of perinatal morbidity and mortality. In this review, developing trends in the treatment of severe preeclampsia are discussed. Expectant treatment of the patient remote from term, anesthesia choices, and delivery route are reviewed. Developing trends in the pharmacological approach to complicated preeclampsia are discussed. New concepts in the treatment of cerebrovascular preeclampsia and hepatic rupture are outlined and reviewed.

Hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome is a form of severe preeclampsia that threatens the gravida and her fetus. In this report, the diagnostic criteria and maternal and fetal risks of HELLP are defined. Prompt recognition and treatment in tertiary centers is emphasized, because the prognosis can be adversely affected by delayed or less than optimal diagnosis and treatment. Management guidelines are offered for treating this disorder. The potential roles of corticosteroids, plasmapheresis, and expectant management are critically evaluated. Subsequent pregnancy outcome, contraception, and preventative strategies are considered.

Our purpose was to determine whether the routine initiation of dexamethasone therapy in patients with postpartum HELLP (hemolysis, elevated liver enzymes, and thrombocytopenia) syndrome produces specific and general therapeutic benefits.

In this retrospective, analytic study the puerperal courses of 43 women with postpartum HELLP syndrome who were treated with dexamethasone were compared with those of 237 similar patients who did not receive corticosteroids. Dexamethasone 10 mg intravenously at 12-hour intervals was given until disease remission was noted in treated patients, at which time up to two additional 5 mg intravenous doses were given at 12-hour intervals.

The two patient groups were similar in regard to mode of delivery, gestational age, parity, and frequency of eclampsia. Compared with control subjects, dexamethasone-treated postpartum patients were more ill with significantly higher (p < 0.05) admission mean arterial blood pressure, higher serum uric acid level, and severe proteinuria. Dexamethasone administration was associated with a more rapid normalization of platelet counts and lactic dehydrogenase values. Most impressive was a clinically significant reduction of indicated transfusion and respiratory therapy, invasive hemodynamic monitoring, infectious or bleeding-related morbidity, and length of postpartum hospital course.

Patients who received dexamethasone for postpartum-onset HELLP syndrome experienced a shorter disease course, faster recovery, less morbidity, and less need for other interventionist therapy compared with patients with HELLP syndrome who did not receive dexamethasone.

The case of a patient with HELLP syndrome, who was unresponsive to supportive management but successfully treated with plasma exchange, is presented. The significance of plasma exchange in the treatment of HELLP syndrome is discussed.

Our purpose was to investigate the postpartum use of plasma exchange in patients considered to have atypical preeclampsia-eclampsia manifested as persistent HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome with or without evidence of other organ injury.

During a 10-year period, 18 patients with HELLP syndrome were treated post partum with single or multiple plasma exchange with fresh-frozen plasma. Each patient was entered into the clinical trial either because of persistent evidence of atypical preeclampsia-eclampsia as HELLP syndrome > 72 hours after delivery (group 1) or with evidence of worsening HELLP syndrome at any time post partum in association with single- or multiple-organ injury (group 2). All procedures were performed with the IBM 2997 Cell Separator (IBM, Cobe Laboratories, Inc., Lakewood, Colo.) system. Maternal and perinatal outcomes were the main outcomes studied.

In the absence of other disease conditions, the 9 patients in group 1 with persistent postpartum HELLP syndrome complicated only by severe clinical expressions of preeclampsia-eclampsia responded rapidly to one or two plasma exchange procedures with few complications and no maternal deaths. In contrast, in the 9 patients of group 2 with HELLP syndrome presentations complicated by other organ disease, the response to plasma exchange was variable and there were two deaths in this group.

The current series of patients details the successful postpartum application of plasma exchange therapy for unremitting HELLP syndrome but reveals that a uniformly positive response to this therapy will not always be observed when there is additional single or multiple organ injury.

To explore the potential efficacy of plasma exchange as an ancillary interventive therapeutic tool immediately before or after delivery in the patient with severe preeclampsia/eclampsia and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome.

Two gravidas with complicated severe preeclampsia/eclampsia/HELLP syndrome were treated emergently in the immediate peripartal period with single-volume plasma exchange and fresh frozen plasma fluid replacement using the IBM 2997 Cell Separator.

Despite multiple platelet unit infusions, one primigravida in active labor at 5 cm cervical dilation and 39 weeks' gestation remained at a platelet count of 14,000/microL and began to ooze from her guns. A second primigravida remained obtunded, oliguric, and thrombocytopenic with epistaxis and hematuria following cesarean delivery and platelet transfusions. A single expedited 3-liter plasma exchange procedure reversed the rapidly deteriorating clinical situation for each patient and accelerated recovery from HELLP syndrome. Both patients and progeny suffered no permanent sequelae.

Based on our experience, we believe that the therapeutic modality of plasma exchange with fresh frozen plasma can be employed effectively for the pregnant patient with severe atypical HELLP syndrome that progressively worsens during labor or the early puerperium despite the use of conventional transfusion therapy.

To explore the efficacy of plasmapheresis/plasma exchange as the primary therapy to arrest and reverse the progression of severe preeclampsia with or without HELLP syndrome in order to postpone delivery and improve perinatal outcome in very preterm pregnancies.

In this case series of patients managed over a 4-year period from 1984 to 1987, seven gravidas with severe preterm preeclampsia underwent 1-2 plasmaphereses/plasma exchange procedures using the IBM 2997 Cell Separator with continuous electronic fetal heart rate monitoring (n = 7 patients) and central cardiovascular monitoring (n = 3 patients).

The seven patients (one with HELLP syndrome, six without HELLP) presented between 24 and 30 weeks gestation and, despite plasmapheresis/plasma exchange, the severity of each study subject's preeclampsia persisted without clinically significant improvement. Maternal-fetal deterioration required cesarean delivery in all cases within 48 (in four patients within < 36) hours of therapy. No clinically significant adverse effect of plasma exchange therapy was recorded during cardiovascular and laboratory monitoring; two fetuses developed repetitive late decelerations during exchange despite adequate maternal fluid preload. The only patient with HELLP syndrome developed eclampsia as her third plasma exchange within 25 hours was being initiated. Significant problems with fluid retention and displacement (variable amounts of pulmonary edema, pleural effusions, large volume ascites) were encountered in all patients. Four neonates died (24-27 weeks/438-820 g) and three survived intact (740, 950, and 1,280 g). One mother (case 5) developed end-stage renal disease 21 months postpartum.

The application of plasmapheresis/plasma exchange therapy as described in order to prolong very preterm pregnancies in the undelivered patient with severe preeclampsia/eclampsia with or without HELLP syndrome did not produced encouraging results. Patients in general were exposed to additional medical and surgical risk without a corresponding improvement in perinatal outcome.

Our purpose was to describe the incidence and effects of serious obstetric complications on maternal outcome in pregnancies complicated by HELLP syndrome.

A prospective cohort study was performed on 442 pregnancies with HELLP syndrome managed at this center from August 1977 through July 1992.

Of 437 women who had 442 pregnancies with HELLP syndrome; 309 (70%) of the cases occurred ante partum and 133 (30%) post partum; 149 (11%) developed at < 27 weeks and 80 (18%) at term. Maternal mortality was 1.1% (five patients). Serious maternal morbidity included disseminated intravascular coagulation (21%), abruptio placentae (16%), acute renal failure (7.7%), pulmonary edema (6%), subcapsular liver hematoma (0.9%), and retinal detachment (0.9%). Fifty-five percent of patients required transfusions with blood or blood products, and 2% required laparotomies for major intraabdominal bleeding. Abruptio placentae was strongly correlated with the development of disseminated intravascular coagulation (p < 0.0001), acute renal failure (p < 0.001), and pulmonary edema (p < 0.01). Moreover, there was a strong association between pulmonary edema and acute renal failure (p < 0.0001). There were no differences in laboratory findings between HELLP syndrome before and after delivery; however, women with postpartum HELLP syndrome had significantly higher incidences of pulmonary edema and renal failure.

HELLP syndrome is associated with serious maternal morbidity, especially when it arises in the postpartum period.