hemolysis in aps

OBJECTIVE: Hepatic infarction is a rare complication of pregnancy most often associated with hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. The objective of this review is to identify risk factors, presenting signs and symptoms, methods of diagnosis, and best management practices based on published case reviews. DATA SOURCES: PUBMED and MEDLINE: OVID databases were searched for citations regarding hepatic infarction in pregnancy or the postpartum period since database inception until the study date of December 18, 2023...

Orthopedic implants made from titanium are a popular choice in the medical field because of their remarkable strength-to-weight ratio. Nevertheless, they may not interact well with human blood, resulting in thrombosis and hemolysis. In fact, non-hemocompatibility is believed to be responsible for about 31 % of medical device failures in the US alone, requiring painful and expensive revision surgery. To address this issue, bioactive hydroxyapatite coatings are applied to Ti-6Al-4V implants using thermal spray techniques...

Hematologists are often needed to assist with the management of microangiopathic emergencies in pregnancy. A firm understanding of the diagnosis and management of preeclampsia with severe features, hemolysis elevated liver enzyme and low platelet syndrome, and disseminated intravascular coagulation, which are the most common causes of microangiopathic emergencies, is critical. However, being able to consider when other microangiopathic emergencies (acute fatty liver of pregnancy, congenital and acquired thrombotic thrombocytopenic purpura, complement mediated microangiopathy, antiphospholipid syndrome) should be considered is imperative...

RATIONALE: Thrombotic microangiopathy (TMA) is a spectrum of rare diseases characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ damage. Differentiating pre-eclampsia, HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome and atypical hemolytic uremic syndrome (aHUS) during pregnancy may be diagnostically challenging yet important as the treatment pathways differ. Most cases of aHUS are associated with dysregulation of the complement alternative pathway, for which current guidelines recommend prompt treatment with complement C5 inhibitor to prevent chronic sequelae...

Antiphospholipid syndrome nephropathy includes a variety of histological lesions, including thrombotic microangiopathy, which is not included among the diagnostic criteria of antiphospholipid syndrome. Whereas in secondary antiphospholipid syndrome, e.g. to systemic lupus erythematosus, there is emerging evidence of a benefit from complement blockade with eculizumab, optimal treatment of primary antiphospholipid syndrome-associated thrombotic microangiopathy is currently unknown. We report the case of a 36-year-old male patient with primary antiphospholipid syndrome-associated thrombotic microangiopathy, presenting with a clinical picture of atypical hemolytic-uremic syndrome with frequent relapses, treated with eculizumab (four 900 mg weekly doses followed by 1200 mg fortnightly infusions) leading to resolution of hemolysis, long-term remission and partial kidney function recovery (peak serum creatinine 3...

Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant clonal hematopoietic disorder characterized by the lack of glycosylphosphatidylinositol-anchored proteins (GPI-APs) as a consequence of somatic mutations in the phosphatidylinositol glycan anchor biosynthesis class A ( PIGA ) gene. Clinical manifestations of PNH are intravascular hemolysis, thrombophilia, and bone marrow failure. Treatment of PNH mainly relies on the use of complement-targeted therapy (C5 inhibitors), with the newest agents being explored against other factors involved in the complement cascade to alleviate unresolved intravascular hemolysis and extravascular hemolysis...

BACKGROUND: Antiphospholipid syndrome (APS), particularly the catastrophic antiphospholipid syndrome (CAPS), is one of the rare causes of thrombotic microangiopathy (TMA). CAPS is the most severe form of APS, especially when accompanied by complement dysregulation, causes progressive microvascular thrombosis and failure in multiple organs. In this report, a case of CAPS with TMA accompanied by a genetic defect in the complement system is presented. CASE: A 13-year-old girl was admitted to the hospital with oliguric acute kidney injury, nephrotic range proteinuria, Coombs positive hemolysis, refractory thrombocytopenia, a low serum complement C3 level and anti-nuclear antibody (ANA) positivity...

In the emergency room, patients with anemia and thrombocytopenia are common. Although these findings can often be explained by the medical situation, thrombotic microangiopathy is an important differential diagnosis. In this case, occlusion of the smallest vessels consequently leads to functional impairment of the affected organs. Patients generally present with symptoms of organ dysfunction, e.g., in the kidney or brain. Characteristically, Coombs-negative fragmentation of erythrocytes with hemolysis occurs in the area of the occluded vessels...

Pregnancy after simultaneous pancreas and kidney transplantation (SPKT) carries a high risk of maternal and fetal complications. We report the case of a 39-year-old woman with three consecutive pregnancies with favorable outcomes after SPKT. Within the first year of SPKT, the patient had a spontaneous pregnancy. At 32 weeks of gestation, she underwent an emergency cesarean section (CS) due to severe preeclampsia and HELLP syndrome. The infant was of average birth weight and was transferred to the neonatal intensive care unit for further management...

The most common thrombotic microangiopathy (TMA) of pregnancy is the well-recognized syndrome of preeclampsia with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. However, rare TMAs, including thrombotic thrombocytopenic purpura, complement-mediated hemolytic-uremic syndrome, and catastrophic antiphospholipid syndrome, may occur during pregnancy or postpartum and present with features similar to those of preeclampsia with severe features. Early recognition and treatment of these infrequently encountered conditions are key for avoiding serious maternal morbidities with long-term sequelae and possible maternal or fetal death...

Thrombotic complications are a hallmark of antiphospholipid syndrome (APS). These vascular - arterial, venous, and/or small vessel - complications are well described and known to hematologists and healthcare providers caring for patients with this disease. In this review, we shed light on other hematological manifestations of the disease, including bleeding, thrombocytopenia, autoimmune hemolytic anemia, and thrombotic microangiopathy syndromes. While these manifestations are not bona fide clinical criteria for the diagnosis of APS, they frequently interact and contribute to the complexity of clinical management of APS...

Thrombocytopenia is a platelet count of less than 150 × 103 per μL and can occur from decreased platelet production, increased destruction, splenic sequestration, or dilution or clumping. Patients with a platelet count greater than 50 × 103 per μL are generally asymptomatic. Patients with platelet counts between 20 and 50 × 103 per μL may have mild skin manifestations such as petechiae, purpura, or ecchymosis. Patients with platelet counts of less than 10 × 103 per μL have a high risk of serious bleeding...

Thrombocytopenia, defined as platelet count ＜150×109 /L, is frequently observed by physicians during pregnancy, with an incidence of approximately 10% of all pregnancies. Most of the cases of thrombocytopenia in pregnancy are due to gestational thrombocytopenia, which does not confer an increased risk of maternal bleeding. However, because other causes can be associated with life-threatening events, such as severe bleeding, that can affect to maternal and fetal outcomes, differentiating other cause of thrombocytopenia, which includes preeclampsia, HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome, acute fatty liver of pregnancy, immune thrombocytopenia, hereditary thrombocytopenia, antiphospholipid syndrome, thrombotic thrombocytopenic purpura, and atypical hemolytic uremic syndrome, is important...

Autoimmune disorders are often associated with low platelet count or thrombocytopenia. In immune-induced thrombocytopenia (IIT), a common mechanism is increased platelet activity, which can have an increased risk of thrombosis. In addition, or alternatively, auto-antibodies suppress platelet formation or augment platelet clearance. Effects of the auto-antibodies are linked to the unique structural and functional characteristics of platelets. Conversely, prior platelet activation may contribute to the innate and adaptive immune responses...

OBJECTIVES: Serum indices included in clinical chemistry instruments are widely used by laboratories to assess the quality of samples. Instruments that report quantitative results allow an evaluation of their diagnostic performance in a similar way to other biochemical tests. The Spanish Society of Laboratory Medicine (SEQC-ML) launched a monthly External Quality program of serum indices in 2018 using three lyophilized materials of simultaneous annual distribution. We present the results of the first three years of the program...

Cytopenias in systemic lupus erythematosus (SLE) require clinical and laboratory workup and bone marrow (BM) examination to determine the cause and for appropriate patient management. Common causes include an increase in SLE activity, immune-mediated hemolysis, iron deficiency, antiphospholipid antibody syndrome, infection, or the effect of medications. We retrospectively evaluated the clinical and laboratory findings of patients with SLE and cytopenias who had undergone BM studies to determine the indicators of malignancy...

Acute neurological problems are significant disorders of pre- and postpartum period in women. We analyzed the magnetic resonance imaging (MRI) profile of patients presenting with peripartum encephalopathy over 2 years. Of 51 patients, MRI was abnormal in 40 patients and normal in 11 patients. Posterior reversible encephalopathy (13/40), cerebral venous thrombosis (6/40), and postpartum angiopathy (3/40) are the three most common causes of peripartum encephalopathy as identified in MRI. The other less common but important etiology include HELLP (Hemolysis, Elevated Liver enzymes and Low Platelet) syndrome (2), osmotic demyelination (2), antiphospholipid syndrome (2), tubercular meningitis/cerebritis (3), pituitary hyperplasia with hemorrhage (2), postictal edema (2), cerebellitis (1), transient splenial lesion (1), and changes of old trauma and stroke (one each)...

SARS-CoV-2 has been reported as a possible triggering factor for the development of several autoimmune diseases and inflammatory dysregulation. Here, we present a case report of a woman with a history of systemic lupus erythematosus and antiphospholipid syndrome, presenting with concurrent COVID-19 infection and immune thrombotic thrombocytopenic purpura (TTP). The patient was treated with plasma exchange, steroids, and caplacizumab with initial good response to therapy. The course of both TTP and COVID-19 disease was mild...

Purpose: Purpose was to improve the skin compatibility and permeability of alpha phellandrene through an ethosomal gel formulation for the treatment of gout; as the oral use of the drug is reported to cause gastrointestinal disturbances and toxicities. Methods: Alpha phellandrene loaded ethosomal formulation (APES) was prepared by cold method for the treatment of gout. APES were loaded into carbopol gel (APEG) by dispersion method. Physico-chemical characterizations of the APES were done by dynamic light scattering (DLS), transmission electron microscopy (TEM), Fourier-transform infrared spectroscopy (FTIR) etc...

Platelets play such an important role in the process of thrombosis that patients with thrombocytopenia generally have an increased risk of bleeding. However, abnormal thrombotic events can sometimes occur in patients with thrombocytopenia, which is unusual and inexplicable. The treatments for thrombocytopenia and thromboembolism are usually contradictory. This review introduces the mechanisms of thromboembolism in patients with different types of thrombocytopenia and outlines treatment recommendations for the prevention and treatment of thrombosis...