Haemoglobinuria R82.3

In 1853, Teichmann was the first to detect hemoglobin as hydrochloric hematin in urine (Guder 1989). In 1858, Heller described a test for the detection of hemoglobin in urine by alkalizing and heating the urine. The benzidine assay still in use today was developed in 1904 by R. and O. Adler (Guder 1989) and three years later the conception of the test strip still in use today was developed (Hofmann 2001).

Hemoglobinuria is the appearance of hemoglobin in the urine. This occurs as a result of intravascular hemolysis (Herold 2022). Hemoglobinuria occurs when the plasma hemoglobin level is > 150 mg / dl (Abdulla 2021).

The causes of hemoglobinuria can be prerenal, renal and postrenal:

- 1. prerenal hemoglobinuria:

Here, hemolysis can occur in vivo and in vitro (Gressner 2019).

Intravascular hemolysis is found in:

- incompatibility after transfusion

- mechanical destruction of erythrocytes (Kasper 2015) e.g. after long marching as marching hemoglobinuria (Herold 2022)

- microangiopathic hemolytic anemia, e.g., in patients with prosthetic heart valves (Kasper 2015)

- in hemolytic crises in the context of hemolytic anemia

- in the case of soap abortion (Herold 2022)

- 2. renal hemoglobinuria:

This may result from bleeding in the tubular system in the presence of hypotonic urine as occurs, for example, in toxic damage to the tubular system, tuberculosis of the kidney, or renal carcinoma (Gressner 2019).

- 3. postrenal hemoglobinuria:

This can result from bleeding into the urinary tract with subsequent hemolysis during passage into the bladder or urine specimen in vitro. Another possibility of hemoglobinuria is contamination with blood from the vagina or, in men, from wounds on the external genitals (Gressner 2019).

Hemoglobinuria occurs when serum haptoglobin capacity is exhausted or the tubular threshold for hemoglobin is exceeded (Scheurlen 1977).

Commercial rapid urine tests are not sufficient for the diagnosis of hemoglobinuria, as they detect both free hemoglobin and hemoglobin bound to erythrocytes (Piechota 1999). Hemoglobin can be detected from a concentration of 1 ml / l urine (Gressner 2019).

The differential diagnostic differentiation between erythrocyturia and hemoglobinuria is only possible by urine sediment (Piechota 1999).

Signs of hemolysis can be detected by:

- LDH elevated

- serum iron significantly elevated

- indirect bilirubin elevated

- Reticulocytes increased

- Hemoglobin decreased

- Erythrocytes decreased

- hematocrit decreased

In addition, erythrocyte morphology plays a role (Knoche 2019).

- Paroxysmal nocturnal hemoglobinuria

This is a rare hereditary disorder (Siegenthaler 2005).

- Hematuria

Hematuria is the appearance of erythrocytes in the urine. Differentiation is by qualitative and quantitative microscopic detection (Bolenz 2018).

In hematuria, erythrocytes are detected in the urine, whereas in hemoglobinuria, hemoglobin not bound in erythrocytes is detectable (Scheurlen 1977).

- Artificial hemoglobinuria.

The number of hemoglobinurias due to subsequent deterioration of erythrocytes is very large. From there, the value of the significance of hemoglobinuria has decreased (Gressner 2019).

Depending on the cause, hemoglobinuria can lead to:

- acute renal failure

- anaphylactic shock

- cardiovascular arrest (Rump 2003)

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