hemoglobinuria

hemoglobinuria /he·mo·glo·bin·uria/ (he″mo-glo″bĭ-nu´re-ah) free hemoglobin in the urine.hemoglobinu´ric

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march hemoglobinuria  that seen after prolonged exercise.

paroxysmal cold hemoglobinuria  an autoimmune or postviral disease marked by episodes of hemoglobinemia and hemoglobinuria after exposure to cold, caused by complement-dependent hemolysis due to Donath-Landsteiner antibody.

paroxysmal nocturnal hemoglobinuria  (PNH) a chronic acquired blood cell abnormality with episodes of intravascular hemolysis and venous thrombosis.

toxic hemoglobinuria  that caused by ingestion of a poison.

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he·mo·glo·bi·nu·ri·a (hm-glb-nr-)

n.

The presence of free hemoglobin in the urine.

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hemo·globi·nuric adj.

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hemoglobinuria

[-oo͡r′ē·ə]

Etymology: Gk, haima + L, globus, ball; Gk, ouron, urine

abnormal presence in the urine of hemoglobin that is not attached to red blood cells. Hemoglobinuria can result from various autoimmune diseases or episodic hemolytic disorders. It can be diagnosed by using a dipstick reagent that is sensitive to free hemoglobin. Kinds of hemoglobinuria include cold hemoglobinuria, march hemoglobinuria, and nocturnal hemoglobinuria. Also spelled haemoglobinuria. Also called toxic hemoglobinuria. -hemoglobinuric, adj.

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hemoglobinuria

the presence of free hemoglobin in the urine. In true hemoglobinuria the protein has originated from erythrocytes hemolyzed within the vascular system. In false hemoglobinuria it comes from erythrocytes in the urine that have hemolyzed there, leaving red cell envelopes in the urinary sediment. In both forms the urine is colored dark red to almost black.

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bacillary hemoglobinuria

is an acute, highly fatal toxemia of cattle and sheep caused by Clostridium haemolyticum (C. novyi type D). It is characterized by hemoglobinuria, jaundice and sudden death with large, usually single infarcts in the liver.

cold hemoglobinuria

intravascular hemolysis occurs in the blood vessels in the intestinal wall immediately after the calf, rarely an adult, has drunk a large amount of very cold water after a period of deprivation. Transient hemoglobinuria may occur. See also cold anemia.

false hemoglobinuria

when lysis of erythrocytes occurs after the urine is passed.

postparturient hemoglobinuria

see postparturient hemoglobinuria.

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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, ↓ T_1/2 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