What Is Hemoglobinuria?

Hemoglobinuria refers to the phenomenon that urine contains free hemoglobin without red blood cells, or only a few red blood cells and contains a large amount of free hemoglobin. Reflects more than normal hemolysis in the blood vessels. Because the hemoglobin content in the urine varies, the urine color can be red, strong tea, and in severe soy sauce. Patients can show different symptoms due to different causes, such as hemoglobinuria in patients with paroxysmal nocturnal hemoglobinuria, which is easy to appear for the first time in the morning. Broad bean disease has a history of eating broad beans or occurs during the broad bean flowering season. Severe hemolysis is often accompanied by anemia, jaundice, liver and spleen. The cause is treated according to the primary disease causing hemoglobinuria.

Basic Information

English name

Hemoglobinuria

Visiting department

Nephrology

Common causes

Hemolysis in the urinary tract, renal infarction, intravascular hemolysis, etc.

Common symptoms

Urine color is red or dark brown, soy sauce color when severe; severe hemolysis is often accompanied by anemia, jaundice, liver and spleen

Causes of hemoglobinuria and common diseases

Hemolysis in the urinary tract

If the relative density of urine is less than 1.006, the red blood cells are dissolved in the urine, and the urine color is red, which is called pseudohemoglobinuria.

2. Renal infarction

Hemolysis occurs in the infarcted area. This type of hemoglobinuria is characterized in that hemoglobin bound to globin and free hemoglobin in the blood are normal, and true hemoglobinuria caused by intravascular hemolysis is easily distinguished.

3. Intravascular hemolysis

It is the most important and common cause of hemoglobinuria, as follows:

(1) Hemolytic anemia caused by birth defects of red blood cells.

(2) Blood group incompatibility: blood transfusion reaction.

(3) Bacterial infection: seen in sepsis and infective bacterial endocarditis; protozoan infection is common in malaria, which is also called black urine fever.

(4) Hemolysis caused by drugs and chemical agents: such as quinine, quinidine, chlorpromazine, phenacetin and other drugs. Chemical preparations and heavy metal salts are commonly phenylhydrazine, nitrobenzene, aniline, arsenic, hydrogen arsenide, lead, etc.

(5) Intravascular hemolysis caused by animal and plant factors: seen in bites by poisonous snakes and poisoned by mushrooms.

Hemoglobinuria test

Laboratory examination is of decisive value for determining the existence and cause of hemolytic anemia. Corresponding special tests such as acid hemolysis, hot hemolysis, cold hemolysis, and erythrocyte shock test are performed according to the clinical possibility to determine the diagnosis of specific diseases. The red blood cell fragility test can be used as a preliminary check. Except for anemia, the increase in reticulocytes is characteristic. Juvenile red blood cells appear in the surrounding blood. The red series of bone marrow examination is active, while the granulose series and megakaryocyte series are normal.

Differential diagnosis of hemoglobinuria

Paroxysmal nocturnal hemoglobinuria

It is a chronic hemolytic anemia with acquired red blood cell intrinsic defects. The occurrence of hemoglobinuria (soy sauce-like urine) is directly related to sleep (not limited to nighttime sleep). A positive acid hemolysis test (Ham test) is of diagnostic significance. The RouS test was positive (ROuS test). The urine sediment showed blue hemosiderin particles after iron staining. Hot hemolysis test, sugar water test is positive for reference.

2. Broad bean disease

It is congenital hereditary erythrocyte deficiency of 6-phosphate glucose deaminase (G-6-PD). The disease appears to be debilitating, jaundice, and soy-colored urine 1 to 2 days after eating broad beans or inhaling broad bean pollen. Confirmation depends on laboratory tests. The commonly used diagnostic methods are: G6-PD activity measurement (the most reliable) glutathione stability test red blood cell Heinz body count leucresol blue dye reduction test methemoglobin reduction test .

3. Paroxysmal cold hemoglobinuria

Refers to the hemoglobinuria of patients with whole or local hand soaking in cold water or eating cold drinks. The laboratory diagnosis is based on a positive hemolysin test (Dang-Lan test) and a direct anti-human globulin test, which is an acquired acquired hemolytic anemia.

4. Hereditary spherocytosis

It is a genetic chronic hemolytic anemia. The diagnosis is based on hemoglobinuria, red blood cells are spherical, jaundice, splenomegaly, and anemia. The main laboratory tests are increased red cell fragility. A family history can help diagnose.

5. Thalassemia

Mainly distributed along the Mediterranean, Italy, Greece and other countries, China's Zhejiang Province also occurs. It is a genetic defect caused by changes in the chemical composition of hemoglobin molecules. Except for the diagnosis in the family of one of the parents, the diagnosis is mainly based on laboratory tests.

6.Malaria

Also known as black urine fever, mosquitoes bite malaria patients and bring malaria parasites into healthy people. Therefore, they are more common in summer and more common in the south than in the north. In addition to the urine being red, brown, and black, the diagnosis is mainly based on the seasonal, regional, and history of contact with malaria patients and the history of blood transfusion.

7. Myoglobinuria

Due to degeneration of muscle tissue, extensive damage to inflammatory muscle tissue, and metabolic disorders, myoglobin exudates from the damaged muscle tissue. Myoglobin has a small molecular weight and is easily excreted from the kidney to cause myoglobinuria. People with large muscle damage such as burns.

Hemoglobinuria treatment principles

According to the different causes of hemoglobinuria, symptomatic treatment is given.