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]