Definition

Affection in the course of which the cooling is a triggering cause or obstructing.

Classification

Example of pathologieL’ essential paroxysmal hemoglobinuria or frigore (English paroxysmal cold haemoglobinuria), also called an obsolete term: Harley, is a condition that occurs in young subjects, accompanied by symptoms due to cooling and with access to hemoglobinuria (presence of hemoglobin in the urine) preceded by chills with fever. The patient also presents crises of anxiety and aches. Analyses highlight, in the serum (the fluid part of blood), a variety of protein: hemolysin. It is a type of antibody (immunoglobulin) of type G (IgG) with the capacity to destroy red blood cells (erythrocytes of Group P). The specialists call it autoantibodies cold anti-P. This particularly rare pathology currently corresponds to a variety of autoimmune hemolytic anemia (a condition in which the patient produces antibodies against its own tissues).More specifically this disease which also has the name of Dressler disease or Donat-Landsteiner syndrome is a chronic disease characterized by hemolysis or the presence of hemoglobin in the urine (hemoglobinuria) that occurs after a general cooling. On the other hand patients have painful episodes of diffuse tingling associated with a rise in temperature.Hemoglobinuria and hemolysis that is the destruction of red blood cells within the vessels is the result of a reaction antibody-Antigen due to Donat-Landsteiner antibody which is a “cold” autoantibodies with the ability to set on red blood cells at low temperature, resulting in hemolysis (destruction of the red blood cell), hemolysis which complement is also as soon as the body warms up to 37 °. A complement is each of the factors involved in cascade, by developing the activity of antibodies. This antibody that also called agglutinin or hemolysin two-phase is really a ox40 IgG directed against the P antigen which is located on the surface of red blood cells (erythrocytes). This reaction of immune dysfunction involves this autoantibodies and particularly in certain infections by viruses such as measles, chickenpox, or mumps etc. During tertiary syphilis (for which the serology is falsely positive) it is also possible to see a similar reaction. In certain cases the action of agglutinin appears without known exactly why, it is idiopathic forms. Idiopathic refers to a disease that exists by itself, out of any other disease state (consequences of the disease) set. Patients have access to hemoglobinuria which are triggered when they are exposed to cold or when they have a fever or after the occurrence of a hot, painful episode or a variable injury. The episodes are then followed by a little jaundice (slight jaundice) and hemolytic anemia. If the patient is in a warm atmosphere access then appears. Between episodes there is no special disruption and evolution is spontaneously favorable. There, nevertheless sometimes on palpation of the abdomen of the patient, hepatosplenomegaly i.e. an increase in the volume of the liver and spleen but it is transient. Laboratory tests highlight subsequently normochromic anemia in which red blood cells appear normal without be overloaded in iron. This review also highlights the presence of hemoglobin in the urine and hemoglobin in the blood (hemoglobin). On the other hand, there is transitional hyperbilirubinaemia (transient bilirubin elevation) after the episode. Finally the Donat-Landsteiner is positive. It highlights hemolysis in vitro (laboratory (red blood cells when they are put in contact with the serum of the patient, provided that it is cooled to 0 ° for 30 minutes then then heated to 37 °.)) It is often necessary, to eliminate other diagnoses, syphilis essentially, search that are falsely positive serological reactions for this infection. The treatment is only to avoid the cooling.

Medical analysis

Sometimes urine are coloured in red because of drugs. This is the case among others the phenolphthalein and rifampin. Beet and some candy, among others, containing dyes stain also urine red. For all these reasons the examination of the patient is very important.The conditions in the laboratory, for dosing hemoglobinuria are the following.It is necessary to obtain a urine sample of an amount ranging from 30 to 60 ml collected in a bottle of clean and, where possible, menstrual periods. Indeed the blood from the rules will come to parasitize the results.On the other hand, must be absolutely avoided muscle efforts before collection.The examination will have to put clearly medication.The preview of the urine allows for at the outset to know whether or not there is hemoglobinuria. Indeed, the presence of hemoglobin in the urine results in a red colour because of the pigment from red blood cells: hemoglobin. This hemoglobin is the result of hemolysis of a breakdown or destruction of red blood cells in the urine.The interest of this review is to make a difference with hemoglobinuria of hematuria that is the presence of blood in the urine that can possibly be the result of a urinary tract infection or impairment of renal function.The results, after this examination are the following: either it is found an absence of hemoglobinuria (step of hemoglobin in the urine).Either there is an intravascular hemolysis with a significant increase of hemoglobin in plasma that is the fluid part of blood.Free hemoglobin in plasma (hemoglobin) varies between 5 mg per decilitre and 50 mg per litre in equivalence: 3.1 millimolles per litre.Plasma free hemoglobin increases in case:

• Intravascular hemolysis.
• Toxic acute hemolytic anemia.
• Transfusion incompatibility.
• Severe bilieuse fever.
• Of paroxysmal hemoglobinuria.
• Of G6PD deficiency.
• In the thalassemias.

On the other hand there is an another precaution in what concerns hemolysis in hypotonic urine. That is why one has to measure urine osmolarity. The term osmosis is the diffusion between two fluids separated by semipermeable walls. In other words the concentration of the urine is important to know also.The presence of Myoglobin in the distinction with hemoglobinuria may be by a broadcast and the presence of bilirubin in the urine. This is also important to know.

Medical technique

Ehrlich is a test designed to show, in vivo (on the individual itself) capacity of hemolysis of plasma for its own red blood cells in paroxysmal hemoglobinuria was frigore. These tests are carried out on the finger of the patient at the level of which the movement by positioning is a link and then cooling this finger by placing it in ice water was arrested, this during a quarter of an hour. It warms then again for a quarter of an hour, the finger in warm water. The recovery of blood obtained by pricking the level of this finger shows that obtained blood is in part a party lacquered and serum is tinted in rosé. Rosenbach test is to measure hemoglobin before and after having dipped a hand in cold water and this for five minutes. In the presence of paroxysmal hemoglobinuria afrigore obtained a different figure after the cooling that higher than noted before immersion (when hand diving in the water).

Differential diagnosis

Hemoglobinuria can appear in other conditions.Severe bilieuse fever is a serious event occurring in the Plasmodium falciparum malaria. This condition occurs in countries with a very intense endemic and subjects that were previously not affected by malaria and of which the Organization has been weakened. Severe bilieuse fever is the result of a brutal destruction of red blood cells within the vessels (intravascular hemolysis) manifested by the appearance of chills, fever high in which the patient is prostrate (immobilized and elongate the face against the floor). These patients present to vomiting and jaundice (jaundice). Laboratory tests show anemia and the presence of hemoglobin in the urine (hemoglobinuria) which might lead to the emergence of an anuria (judgment of secretion of urine) and a lack of functioning of the renal filtration. Evolution is sometimes fatal. It seems that quinine plays a role promoting in this condition for some teams specialized in Parasitology and infectious diseases.The paroxysmal nocturnal hemoglobinuria is also called disease or syndrome Marchiafava-Micheli and HPN. It is a disease characterized by the onset of crises of intravascular haemolysis occurring during sleep. They are the result of abnormal sensitivity of red blood cells to the action of the add-in. The main sign of the paroxysmal nocturnal hemoglobinuria is the appearance of urine dark when the patient stands up in the morning.It is a disease genetic is the result of mutation of the PIG – A gene (located on the XP 22 locus). A complement is an element, specifically a factor involved in cascade and which plays an important role in the development of antibodies. This sensitivity is usually increased to complement C3 and the results of an abnormality of the membrane. The paroxysmal nocturnal hemoglobinuria is even more serious that the disease depends on the amount of abnormal red blood cells to the amount of healthy red blood cells (nons achieved). On the other hand any activation system involving the complement leads to intravascular hemolysis course on abnormal red blood cells. This is the case among other infection that leads to the occurrence of this phenomenon. It is the administration of iron, the injection of some vaccines and transfusion of blood. This mechanism can also occur during the menstrual period or during pregnancy. The presence of hemoglobin in the urine is the result of hemolysis of red blood cells burst. Losses of hemoglobin in the urine are successful, in the course of this disease, iron loss.This pathology concerns more specifically the men and women and most often individuals whose age is between 20 and 30 years. This disease results in the emergence of crises of hemoglobinuria night with chills, malaise and sometimes belly aches (what in Gastroenterology and Hematology specialists called painful abdominal symptoms). The patient has on the other hand aching. The evolution of the paroxysmal nocturnal hemoglobinuria shows the appearance of severe hemolytic anemia characterized by intense pallor and a near phenomenon of jaundice (subictère). The examination of the patient highlights an increase in volume of spleen (splenomegaly). At the same time it is observed sometimes a deficiency of iron despite the saturation of some organs i.e. the concentration of certain organs such as the kidney hemosiderin. The hemosiderin is a pigment that is not soluble in the blood and which contain a variety of railway existing in the form of ferric hydroxide and constituting a way to store iron in the body representing about 30% of the total iron has an individual. The laboratory, in paroxysmal nocturnal hemoglobinuria, highlights outside the presence of dark urine in the morning, anemia laboratory and normochromic (aspect and red blood cells which are normal iron concentration) usually accompanied a decline in the number of white blood cells and a decline in the number of platelets (thrombocytopenia, pancytopenia). Hematology (Hematologists) professionals perform test of Ham-Dacia is to hemolysis of the blood of the patient, blood is acidified by carbon dioxide and then put in an oven at a temperature of 37 ° and this for an hour. Finally laboratory tests include the search for a hémosidérinurie (presence of hemosiderin in urine) that is constant as well as evidenced by a reduction in alkaline phosphatase staining leucocyte (PAL).Some complications are likely to occur in paroxysmal nocturnal hemoglobinuria. It is a certain predisposition or arterial and venous thrombosis (blood clot from sometimes seriously disrupt the General blood occurred). It is the most dreaded complication of this disease which sometimes results in the death of the patient. The second complication is the occurrence of the disease of Budd-Chiari. It is a rare condition that usually occurs in young subjects (between 30 and 40 years) and characterized by a blockage of the hepatic vein or veins that lead the liver to the inferior vena cava. Other rare complications are the occurrence of an osteofibrosis (“elasticity” loss of the bone marrow) and aplastic anemia see leukemia. Outside these complications the prognosis of this disease is good because survival is prolonged. The treatment involves the administration of cortisone (corticosteroid) as e.g. prednisolone 10 to 50 mg daily. Unfortunately the effect of this treatment is often transient. In some patients it is necessary to transfuse red blood cell-based sockets that were removed (red cells, leucocyte and déplamatisés) plasma and leukocytes. Must be on the other hand avoid plasma, or may contain substances complement C 3, having the property trigger a breakdown of red blood cells (acute hemolytic access). Infusion of 1000 ml of solution of dextran molecular weight is low, has been proposed by some medical teams specialized in hematology, in episodes of hemolysis. Iron supplementation for testing (decrease of red blood cell iron concentration) has also been proposed. It must be done with caution and giving progressive doses. Anticoagulants are sometimes profitable for Budd-Chiari disease or other problems of coagulation leading to the formation of intravascular thrombosis.