Clinical Study of a Novel Hemolysis Point of Care Test at an Emergency Department.
HemolysisBlood Specimen CollectionThis study aims to investigate the outcome of hemolysis frequency when a hemolysis point of care test is introduced in an emergency department.
Clinical Characteristics and Treatment Outcomes of Patients With Autoimmune Hemolytic Anemia
Autoimmune Hemolytic AnemiaAutoimmune hemolytic anemia is characterized by shortened red blood cell survival and a positive Coombs test. The responsible autoantibodies may be either warm reactive or cold reactive. The rate of hemolysis and the severity of the anemia may vary from mild to severe and life-threatening. Diagnosis is made in the laboratory by the findings of anemia, reticulocytosis, a positive Coombs test, and specific serologic tests. The prognosis is generally good but renal failure and death sometimes occur, especially in cases mediated by drugs.
Isoagglutinins in the Development of IVIG-associated Hemolysis
ImmunoglobulinsIntravenous1 morePatients at high risk of IVIG-associated hemolysis (defined as receipt of a 28-day cumulative dose of ≥ 2 g/kg, adjusted for ideal body weight, and non-O blood group) will be prospectively monitored using a standardized protocol for signs of hemolysis, and will be undergo additional testing for variables that have been hypothesized to increase the risk of hemolysis. The goal of the study is to define the incidence and dynamics of IVIG-mediated hemolysis and identify patient and product-related factors that may predict which patients are especially at risk.
Evaluation of Toxicity From Stem Cell Transplant
Organ DysfunctionHemolysisThis study will try to determine what causes toxic side effects of stem cell transplantation, such as increased blood pressure, increased heart rate, decreased kidney function and abnormal heart rhythms. Stem cells are used to treat various diseases, including cancer, aplastic anemia and sickle cell disease. The cells may be given fresh to the patient or they may be preserved first with a chemical called DMSO and frozen for later use. Some stem cell transplant procedures include infusion of red blood cells along with the stem cells. This study will examine whether side effects of stem cell transplants are associated with the DMSO preservative in frozen cells or with hemoglobin (a protein released from defrosted red blood cells) or neither of these factors. Healthy volunteers and patients scheduled to receive a stem cell transplant may be eligible for this study. Candidates must be between 10 and 80 years of age. Transplant patients will undergo a stem cell transplant. The cells are infused through a catheter placed in a vein for the procedure. Depending on the patient s requirements, the infusion may or may not include red blood cells and may or may not contain DMSO. Healthy volunteers undergo a 4-hour saline infusion. The saline (water mixed with salt) is infused through a catheter (plastic tube) placed in a vein in the arm. In addition, all participants have the following tests and procedures: Heart monitoring: Healthy volunteers wear a portable heart monitor, attached to the chest using four stickers, for 24 hours starting the morning of the infusion. Transplant patients wear the same device for 48 hours, starting the morning before the infusion. Blood draws and urine collections before, during, just after and the morning after the infusion of saline or stem cells. Heart ultrasound before, during or just after and the morning after the infusion. Peripheral artery tonometry: A small cup is placed on one finger of each hand to measure blood flow in the finger. A blood pressure cuff is inflated around the lower arm and tight pressure is maintained for about 5 minutes.
Evaluation of Potential Predictors of Disease Progression in Participants With Atypical Hemolytic...
Atypical Hemolytic Uremic SyndromeThis was a prospective, open-label study with no participant randomization. Treatment for aHUS was observational and at the discretion of the treating physician. The purpose of this study was to assess disease manifestations of complement-mediated thrombotic microangiopathy (TMA) and evaluate potential clinical predictors of disease manifestations and progression in participants with aHUS with or without eculizumab treatment in the clinical setting.
International Registry and Biorepository for TMA(Thrombotic Microangiopathy)
Thrombotic MicroangiopathyHemolytic Uremic Syndrome1 moreThis registry will collect clinical data and store biosamples (seru, plasma, urine, and DNA) annually from pediatric patients with thrombotic mcroangiopathy
Pyruvate Kinase (PK) Deficiency Global Longitudinal Registry: Patient-Reported Outcomes (PRO)
Pyruvate Kinase DeficiencyPyruvate Kinase Deficiency Anemia3 moreThe primary objective of this study is to understand and characterize the health-related quality of life (HRQoL) and disease burden of adult participants with PK deficiency receiving routine clinical care. This study is an observational (i.e., noninterventional), longitudinal, multicenter, global registry for participants with PK deficiency, a rare nonspherocytic hemolytic anemia. This study will be open for enrollment for 2 years and all enrolled participants will be followed prospectively for up to 96 weeks. Data will be collected from participants who have provided informed consent and authorization pursuant to applicable laws and regulations.
aHUS Observational Long Term Follow-Up
Atypical Hemolytic Uremic SyndromeThere is growing but limited information on the long term clinical status of aHUS patients who have previously received or are continuing to receive treatment with eculizumab. This study is designed to collect clinical data that will provide insight into the long-term outcomes of patients with aHUS.
Clinical and Laboratory Investigation of Humans With Informative Iron or Erythroid Phenotypes
HemoglobinopathiesHemolysis2 moreThis study will examine blood for factors that may cause or prevent diseases involving iron or red blood cells. Iron is an important nutrient for human health that is needed to produce red blood cells. Red blood cells carry oxygen to body tissues. A better understanding of iron and red blood cells may help lead to better treatment of several diseases including anemia. Patients of all ages with red cell abnormalities in the following categories may be eligible for this study: Diseases with deficiency, overload or maldistribution of iron Known red blood cell diseases, such as anemias and hemoglobinopathies Red blood cell diseases of unknown cause, such as hemolysis of unknown cause Red blood cell abnormalities with no overt clinical disease, such as hereditary persistence of fetal hemoglobin Participants undergo the following procedures: Medical history Physical examination Standard medical tests related to the individual's iron or red blood cell condition Blood draw for the following purposes: Testing for syphilis and for the hepatitis B and C, HIV, and HTLV-1viruses, and for a pregnancy test for women who can become pregnant Research purposes. This blood is analyzed for genes, proteins, sugars, and fat molecules.
Exhaled Carbon Monoxide as a Marker of Hemolysis in Sickle Cell Disease- an Exploratory Study
Sickle Cell DiseaseThe investigators propose to evaluate etCO in patients with HbSS, HbSC, and HbS-beta thalassemia during routine clinic visits, and longitudinally. Our goal is to know whether etCO differs amongst subjects with different sickle cell syndrome genotypes, and whether it is a stable marker of hemolytic rate, as reflected in routine labs obtained for clinical care (including total hemoglobin, reticulocyte count, lactate dehydrogenase, and, when sampled, total and direct bilirubin). We hope to establish whether this inexpensive and non-invasive test faithfully reflects hemolytic parameters in sickle cell syndromes.