Brief Report: Plasma Concentrations of Hemopexin, Haptoglobin and Heme in Patients with Various Hemolytic Diseases

Müller‐Eberhard, Ursula; Javid, Jamshid; Liem, H. H.; Hanstein, A.; Hanna, Marwan

doi:10.1182/blood.v32.5.811.811

Cited by 302 publications

(188 citation statements)

References 8 publications

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“…Before TPE, all our patients had highly elevated heme levels compared to stable SCD patients (mean 6 SD, 4.2 6 1.1 mmol/L; range, 0-20 mmol/L) 3 and very low Hp levels, although only Patient 1 also had very low Hx concentrations ( Table 2). This is consistent with previous observation that Hx is never diminished without reduction in Hp 42 and in vitro studies that with oxyHb excess, Hx activity is spared until Hp becomes saturated. 71 Also, some Hx may be recycled after receptor uptake in contrast to Hp being degraded.…”

Section: Discussionsupporting

confidence: 94%

“…[4][5][6][7][8]15,[36][37][38] The chronic hemolysis of SCD continuously releases Hb into the plasma, 39,40 resulting in steady-state depletion of Hp in 50% to 90% and of Hx in 10% to 30% of patients. 3,41,42 Brazilian children with steady-state SS disease (n 5 173) had mean plasma Hp and Hx levels of 8 and 14% of healthy AA control children, respectively, and SC children (n 5 93) likewise had levels of 16 and 39%, respectively. 43 Contributing to depletion, Hp and Hx may be catabolized upon endocytosis without sufficient compensatory increases in biosynthesis.…”

Section: Results: Plasma Concentrations Of Hp and Hxmentioning

confidence: 99%

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Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

et al. 2017

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Section: Discussionsupporting

confidence: 94%

Section: Results: Plasma Concentrations Of Hp and Hxmentioning

confidence: 99%

Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

et al. 2017

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“…Cell-free haemoglobin is rapidly converted to methaemoglobin with liberation of the haem group, which may also elicit cytotoxic and inflammatory-mediated kidney damage (Tracz et al, 2007;Nath & Katusic, 2012). In chronic haemolytic disease states including SCA, the scavengers of free haemoglobin (haptoglobin) and free haem (haemopexin) are consumed resulting in increased circulation of cell-free haemoglobin and haem (Muller-Eberhard et al, 1968).…”

Section: Discussionmentioning

confidence: 99%

Haemoglobinuria is associated with chronic kidney disease and its progression in patients with sickle cell anaemia

et al. 2013

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Summary To evaluate the association between haemoglobinuria and chronic kidney disease (CKD) in sickle cell anaemia (SCA), we analysed 356 adult haemoglobin SS or Sβ° thalassaemia patients from the University of Illinois at Chicago (UIC) and 439 from the multi-centre Walk-Treatment of Pulmonary Hypertension and Sickle Cell Disease with Sildenafil Therapy (PHaSST) cohort. CKD was classified according to National Kidney Foundation Kidney Disease Outcomes Quality Initiatives guidelines. Haemoglobinuria, defined as positive haem on urine dipstick with absent red blood cells on microscopy, was confirmed by enzyme-linked immunosorbent assay in a subset of patients. The prevalence of CKD was 58% in the UIC cohort and 54% in the Walk-PHaSST cohort, and haemoglobinuria was observed in 36% and 20% of the patients, respectively. Pathway analysis in both cohorts indicated an independent association of lactate dehydrogenase with haemoglobinuria and, in turn, independent associations of haemoglobinuria and age with CKD (P<0.0001). After a median of 32 months of follow-up in the UIC cohort, haemoglobinuria was associated with progression of CKD (halving of estimated glomerular filtration rate or requirement for dialysis; Hazard ratio [HR] 13.9, 95% confidence interval [CI] 1.7-113.2, P=0.0012) and increasing albuminuria (HR 3.1, 95% CI: 1.3-7.7; logrank P=0.0035). In conclusion haemoglobinuria is common in SCA and is associated with CKD, consistent with a role for intravascular haemolysis in the pathogenesis of renal dysfunction in SCA.

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“…Muller-Eberhard & Bashore (1970) found that in cases of Rh isoimmunization the ratio of haemopexin to albumin in amniotic fluid fell during severe fetal haemolysis. The concentration of haemopexin in adult and neonatal serum falls when haemolysis occurs for any reason (Muller-Eberhard et al, 1968;Sears, 1969). This is believed to occur as a result of the haemopexin-haem complexes being removed from the serum by the liver (Muller-Eberhard, Liem, Hanstein & Saarinen, 1969).…”

Section: (Ii) Simple Quantitative Studiesmentioning

confidence: 99%

The Nature and Origin of the Soluble Protein in Human Amniotic Fluid

Sutcliffe

1975

Biological Reviews

102

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Summary 1. Amniotic fluid surrounds the human fetus and is separated from the uterus by the amnion, chorion and placenta. The ability to obtain samples of amniotic fluid from women by a simple procedure has encouraged studies on the nature and origin of the fluid, and on its use for the diagnosis of a variety of clinical conditions. The fluid contains cells, which are of fetal origin, and can be grown in a tissue culture. Cyto‐genetic and biochemical analyses can therefore be used to detect chromosomal aberrations and inborn errors of metabolism in the fetus. 2. The supernatant of amniotic fluid contains many of the solutes typical of extracellular fluid. In particular, it contains a wide range of proteins and those which are of fetal origin are likely to be of use in the prenatal diagnosis of fetal disease. This review examines the nature and origin of the soluble protein in amniotic fluid, and discusses the diagnostic uses of the proteins which are of fetal origin. 3. In other mammals, the arrangement of the fetal membranes is different from that in man, and these differences are reflected by changes in the nature of the amniotic fluid. Thus data from other animals have little applicability to man. 4. Electrophoresis and immunoelectrophoresis have established that the major proteins in amniotic fluid are also present in maternal and fetal sera. Their concentrations in the fluid are influenced by their molecular weight and proteins larger than about 2.5 times 106 may be excluded. Towards term, phenotyping studies show that a number of serum proteins in amniotic fluid are of maternal origin. In the case of group‐specific component (Gc) this has been shown to be so throughout pregnancy. Such proteins must enter the fluid by diffusing across either the chorion or the chorionic plate and then the amnion. 5. It has been previously claimed that various serum proteins in amniotic fluid are of fetal origin. For albumin and IgG there are data that strongly support a maternal origin. The evidence on the origin of insulin is inconclusive. The concentration of β2‐microglobulin in amniotic fluid exceeds that in maternal serum and is probably too high also for fetal serum to be its major source. It has a wide tissue distribution and probably enters the fluid from surrounding structures. 6. Alpha‐fetoprotein in amniotic fluid is of fetal origin as it is present in maternal serum at far lower concentrations. It is found in fetal serum, urine and yolk sac, but it is not clear how it enters the amniotic fluid of normal fetuses. The concentrations of Gc and alpha‐fetoprotein have been measured in amniotic fluid and in their sera of origin. The relative concentration of Gc in amniotic fluid was found to be much greater than that of alpha‐fetoprotein and the concentration gradients of these marker proteins can be compared with data for other proteins. In this way further evidence has been obtained that the albumin, α1,‐antitrypsin and transferrin in amniotic fluid are mainly of maternal origin throughout pregnancy. 7. Immunological ...

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Brief Report: Plasma Concentrations of Hemopexin, Haptoglobin and Heme in Patients with Various Hemolytic Diseases

Cited by 302 publications

References 8 publications

Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

Haemoglobinuria is associated with chronic kidney disease and its progression in patients with sickle cell anaemia

The Nature and Origin of the Soluble Protein in Human Amniotic Fluid

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