hemoglobinuria


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hemoglobinuria

 [he″mo-glo″bĭ-nu´re-ah]
the presence of free hemoglobin in the urine. adj., adj hemoglobinu´ric.
march hemoglobinuria hemolysis caused by repeated uncushioned shocks or trauma to some body part, such as in some soldiers on long marches, in marathon runners, and in karate practitioners.
paroxysmal cold hemoglobinuria an autoimmune or postviral disease in which there is a biphasic IgG antibody directed against the P blood group antigen. It is marked by episodes of hemoglobinemia and hemoglobinuria after exposure to cold and is detected by the Donath-Landsteiner test. The condition is treated with prednisone and cyclophosphamide and by protection from exposure to cold.
paroxysmal nocturnal hemoglobinuria (PNH) an acquired blood cell abnormality with proliferation of abnormal red blood cells (PNH cells) that are readily hemolyzed by complement, and episodes of severe hemolysis and thrombosis, particularly of the hepatic veins. It is detected by the ham test. Treatment is with androgens or prednisone and, during thrombotic episodes, with heparin.

he·mo·glo·bi·nu·ri·a

(hē'mō-glō'bi-nyū'rē-ă),
The presence of hemoglobin in the urine, including certain closely related pigments that are formed from slight alteration of the hemoglobin molecule; when present in sufficient quantities, they result in the urine, being colored in shades varying from light reddish-yellow to fairly dark red.
[hemoglobin + G. ouron, urine]

hemoglobinuria

(hē′mə-glō′bə-no͝o′ē-ə, -nyo͝or′-)
n.
The presence of hemoglobin in the urine.

he′mo·glo′bi·nu′ric adj.

hemoglobinuria

Hematology The presence of Hb in the urine which, if of sufficient quantity, colors urine, the intensity of which directly correlates with the quantity of Hb. See Paroxysmal cold hemoglobinuria, Paroxysmal nocturnal hemoglobinuria.
hemolysis Destruction or lysis of RBCs
Hemolysis
Intracorpuscular hemolysis
•  Membrane defects, eg hereditary elliptocytosis, spherocytosis, stomatocytosis and paroxysmal nocturnal hemoglobinuria
•  Metabolic defects, eg G6PD, pyruvate kinase deficiency
•  Abnormal Hbs see Hemoglobin.
Extracorpuscular hemolysis
1º immune reactions, eg autoimmune hemolytic anemia
2º immune reactions, due to
• Infections, eg Bartonella, Clostridia, malaria, sepsis
• Neoplasia, eg lymphoma, leukemias
• Drug reactions due to the 'Innocent bystander' phenomenon (drug-antibody complex activates complement, causing intravascular hemolysis, eg quinidine), hapten-mediated —a protein-bound drug attaches to the red cell membrane, eliciting an immune response when the hapten-protein complex is recognized as foreign, evoking an immune response, eg penicillin acting as a hapten
• Induction of autoimmunity by RBC antigen alterations, eg Rh antigen
Physical, eg thermal, concentrated glycerol due to inadequate washing of frozen blood, bladder irrigation, cardiac valves
Extravascular Less severe, IgG-mediated and does not activate complement, eg Rh, Kell, Duffy Laboratory ↓ haptoglobin, ↓ T1/2 of circulating RBCs, ↑ indirect BR as liver capacity to conjugate BR–ergo direct BR is overwhelmed by massive hemolysis, ↑ LDH, Hb in blood and urine, hemosiderinuria, MetHb and metalbumin, ↑ urobilinogen in urine and feces, ↑ in acid phosphatase, K+, and prostatic acid phosphatase Clin Chem 1992; 38:575; peripheral smears demonstrate anisocytosis, polychromatophilia, nucleated RBCs, basophilic stippling; immune hemolysis is suggested by spherocytes NEJM 2000; 342:722cpc
Intravascular More severe, IgM-mediated and requires complement activation, eg ABO blood groups Laboratory ↑ free Hb  Note: Clinically significant hemolysis is usually detected by hemagglutination, less commonly by hemolysis per se, which detects anti-P,
-P1, -PP1Pk, -Jka, -Lea, occasionally also anti-Leb and -Vel

he·mo·glo·bi·nu·ri·a

(hē'mō-glō'bi-nyūr'ē-ă)
The presence of hemoglobin in the urine, including certain closely related pigments formed from slight alteration of the hemoglobin molecule; indicative of intravascular hemolysis or of bleeding into the urinary tract, with hemolysis there. The urine may be reddish-yellow to dark red.
Synonym(s): haemoglobinuria.
[hemoglobin + G. ouron, urine]

he·mo·glo·bi·nu·ri·a

(hē'mō-glō'bi-nyūr'ē-ă)
Presence of hemoglobin in urine; when present in sufficient quantities, they result in the urine's being colored in shades varying from light reddish-yellow to fairly dark red.
Synonym(s): haemoglobinuria.
[hemoglobin + G. ouron, urine]
References in periodicals archive ?
Mortality Rate and Case Fatality Rate due to Hemoglobinuria: In the sample, mortality occurred due to Hemoglobinuria only.
Patient Preferences for the Treatment of Paroxysmal Nocturnal Hemoglobinuria: Results of a Patient Survey of Ravulizumab (ALXN1210) and Eculizumab, EHA Congress, June 13-16, 2019 Amsterdam, Netherlands, poster presentation, June 14, 2019, 5:30 p.m., abstract PF734.
* In the setting of most liver diseases, hemoglobinuria, increases of primarily unconjugated bilirubin, and otherwise unexplained high LDH measurements are the most useful in diagnosing hemolysis.
Alexion's two lead product candidates, eculizumab and pexelizumab, are currently undergoing evaluation in several clinical development programs, including two Phase III trials of Soliris (eculizumab) for the treatment of paroxysmal nocturnal hemoglobinuria (PNH).
Paroxysmal nocturnal hemoglobinuria is a chronic, progressive, debilitating and life-threatening ultra-rare blood disorder characterized by hemolysis (destruction of red blood cells) that is mediated by an uncontrolled activation of the complement system, a component of the body's immune system.
GlobalData's clinical trial report, "Paroxysmal Nocturnal Hemoglobinuria Global Clinical Trials Review, H2, 2015" provides an overview of Paroxysmal Nocturnal Hemoglobinuria clinical trials scenario.
Soliris, marketed by Alexion Pharmaceuticals, is currently indicated to treat ultra-rare blood disorders, including paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome.
Hemoglobinuria, a characteristic sign of babesiosis, is not mentioned in these early 20th century reports.
(Nasdaq: ALXN), Cheshire, Conn., has completed enrollment of the Phase III SHEPHERD trial of eculizumab in patients with the chronic orphan blood disorder Paroxysmal Nocturnal Hemoglobinuria ("PNH").
Acute renal failure associate with myoglobinuria and hemoglobinuria. In: Brenner BM, Lazarus JM, eds.
Apellis Pharmaceuticals announced the completion of enrollment for its PEGASUS Phase 3 trial assessing the safety and efficacy of APL-2 in patients with paroxysmal nocturnal hemoglobinuria compared to eculizumab.