Hemoglobinuria - Causes, Symptoms, Diagnosis and Treatment
Hemoglobinuria - A number of syndromes which are based on damage intravascular yield erythrocyte hemoglobin outside the vascular bed and urine. The main symptom is hemoglobinuria urine dark color due to the presence therein of oxyhemoglobin. There are arthralgia, fever, vomiting, pale skin, jaundice. Hemoglobinuria diagnosis be confirmed by examining the general analysis of blood and urine, myelogram, for functional and laboratory samples. Taking into account the pathogenic form can be shown hemoglobinuria red blood cell transfusions, use of hormones, anticoagulants, splenectomy, TCM.
Hemoglobinuria
hemolytic anemia occurring with intravascular hemolysis of red blood cells and the appearance of free hemoglobin in the urine. These include paroxysmal nocturnal hemoglobinuria (illness Marchiafava Michele), toxic, sustainer and paroxysmal cold hemoglobinuria. These states, different etiology, combines a massive, acute intravascular hemolysis has developed, accompanied by the release of hemoglobin in the blood plasma and renal overcoming the barrier. In hematology distinguish single (symptomatic) and repetitive (paroxysmal) hemoglobinuria.
In healthy individuals the bloodstream occurs in approximately 10% decay of erythrocytes, and plasma contained 4.1 mg% hemoglobin. In this case the excess of hemoglobin binds to a specific protein haptoglobin to form krupnomolekulyarnyh complex hemoglobin-haptoglobin, does not overcome the kidney barrier. This mechanism prevents hemoglobinuria and renal hemosiderosis. However, the sudden massive breakdown of red blood cells or gipogaptoglobinemii, leading to a decrease in the ability gemoglobinsvyazyvayuschey plasma, hemoglobin overcomes the kidney filter and appears in the urine. In the normal content of haptoglobin, hemoglobinuria develops when the hemoglobin concentration in plasma is greater than 125-135 mg%.
post-transfusion complication of blood transfusion for incompatible group or Rh-affiliation. Marching hemoglobinuria develops after many kilometers of passages (marches), or cross-country loads. This form hemoglobinuria usually diagnosed in physically healthy individuals, more often - soldiers, athletes, travelers. In this case, the destruction of red blood cells is triggered by the load on the foot, but this phenomenon discovered in the years of the First World War, there is still no scientific explanation. Presumably, long and rough mechanical stimulation of the foot sole original cause haemolysis of red blood cells in the capillaries of the field, and then applies to all the bloodstream.
Cold nocturnal hemoglobinuria can develop as a result of long-term cooling of the body (swimming in cold water, exposure to cold air), as well as infectious diseases (influenza, mumps, measles, infectious mononucleosis, malaria, syphilis, anaerobic sepsis). For this form is characterized by the appearance of blood in biphasic hemolysin Donat-Landsteiner, causing complement activation and intravascular hemolysis.
There are other forms of hemoglobinuria -. Alimentary toxic paroxysmal myoglobinuria (gaffskaya disease), traumatic hemoglobinuria with crush syndrome, etc. Transient hemoglobinuria occurs in patients taking iron supplements.
vaccination, blood transfusion, physical activity, surgery.
Paroxysms of haemolysis of red blood cells accompanied by fever, arthralgia, drowsiness, pain in the chest, abdominal and lower back. A sign of the growing iron-deficiency anemia are weakness, jaundice of the skin and mucous membranes. Characteristic enlargement of the liver and spleen. Isolation of dark-colored urine is observed only a quarter of patients with paroxysmal nocturnal hemoglobinuria.
The most dangerous manifestations of paroxysmal nocturnal hemoglobinuria are thrombosis mesenteric vessels, renal vessels, peripheral vessels. Thrombosis of the hepatic veins accompanied by a sharp increase in the size of the liver, progressive ascites, esophageal varices. Permanent gemosiderinuriya often leads to a tubular jade; at an altitude of hemolytic crisis may develop acute renal failure.
Paroxysmal nocturnal hemoglobinuria often develops in patients with aplastic anemia, predleykozom or acute myeloid leukemia, therefore, it requires a complete hematological examination of the patient.
Marching hemoglobinuria
Symptoms march hemoglobinuria usually more worn and develops gradually. Chills are uncommon and increasing temperature. Marked weakness, which may also be a consequence of the overall physical fatigue from forced march. However, this form of indication pathognomonic hemoglobinuria is a dark color of urine. After the cessation of feeling normal, urine lighter load march.
It has been observed that almost all those affected by the march hemoglobinuria, there is a pronounced lumbar lordosis. Independent clinical significance of this form does not have hemoglobinuria.
Cold nocturnal hemoglobinuria
A rare form of hemolytic anemia - paroxysmal cold hemoglobinuria paroxysmal flows. Paroxysmal cold hemoglobinuria usually provoked by hypothermia and hyperthermia accompanied (sometimes up to 40 ° C), shaking chills, nausea and vomiting, abdominal pain. Reveals hepato- and splenomegaly, yellowish skin and sclera, the characteristic color of urine.
Paroxysmal cold hemoglobinuria caused by acute viral infections usually resolves with the underlying disease. When syphilis and malaria, the disease can last for years.
coagulation, blood biochemical parameters (bilirubin, urea, alkaline phosphatase, and others.). To assess the state of blood, especially in the case of pancytopenia in paroxysmal nocturnal hemoglobinuria, requires a bone marrow puncture and research myelogram.
Must be distinguished from hemoglobinuria and hematuria diseases for which it is typical (kidney stones, acute glomerulonephritis), porphyria, autoimmune hemolytic anemia, aplastic anemia.
Treatment hemoglobinuria
Therapeutic tactics in various forms hemoglobinuria defined by a hematologist. Cruising and paroxysmal cold hemoglobinuria usually resolved without special intervention. In the case of chronic autoimmune cold hemoglobinuria appointed corticosteroids and immunosuppressive drugs (cyclophosphamide, azathioprine).
Treatment of paroxysmal nocturnal hemoglobinuria primarily symptomatic: transfusions of red blood cells, the introduction of indirect anticoagulants, oral iron. In hypoplastic bone marrow appointed steroids (prednisone), anabolic drugs (Nerobolum) or androgens, anti-thymocyte immunoglobulin. When expressed hypersplenism may be justified splenectomy. In the absence of the effect of other treatments for paroxysmal nocturnal hemoglobinuria addressed the issue of a bone marrow transplant from a compatible donor for HLA-system.
In order to prevent the development of hemoglobinuria advised to avoid provoking factors: poisoning, intoxication, infectious diseases, excessive physical stress, injuries, etc.
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