Haemoglobinopathy | Hemoglobin

Haemoglobinopathy

Hemoglobinopathy is the umbrella term for diseases that cause changes in hemoglobin. These are genetically predisposed. The best known are sickle cell anemia and thalassemia (divided into alpha and beta thalassemia). These diseases are either caused by a mutation, i.e. a change in the proteins (sickle cell anaemia) or by a reduced production of these proteins (thalassemia).The diseases have in common that they can be of varying degrees of severity and, depending on their severity, lead to mild or severe anaemia or even to an infant not capable of survival.

Hemoglobin in urine

If haemoglobin molecules are found in the blood, this is a sign of an increased death of red blood cells in the bloodstream. This is known as hemoglobinuria. The kidney filters the entire blood stream several times within an hour.

As a rule, however, it does not filter proteins. If erythrocytes die inside the vessels instead of in the spleen, where they are usually broken down, an excessive number of hemoglobin molecules are freely available in the blood. The kidney then filters them out and excretes them in the urine.

Just as hemoglobin gives the blood its red color, so urine turns dark red. Malaria also causes hemoglobinuria. More common, however, are certain types of anemia or errors in transfusions.

If the cause is in the vascular system, haemoglobinuria is often present. It should not be confused with hematuria, where complete blood is found in the urine. The reasons and causes for this are different.

HbA1C

Hemoglobin A1C is a special form of hemoglobin in the human body. It is also known as glycohemoglobin and describes a normal hemoglobin molecule to which glucose is coupled. Every person has a small amount of HbA1C in their blood, and this can be determined by a blood test.

If there is a particularly large amount of sugar in the blood in the form of glucose, the conversion of hemoglobin into its “glycated” form takes place without enzymes. This process is not reversible. Since an erythrocyte survives for an average of eight weeks before it is broken down, the amount of HbA1C can be used to estimate the amount of sugar in the blood over the last few weeks.

The HbA1C level is therefore considered to be the blood sugar memory and is used in medicine. Patients who have a known sugar disease diabetes mellitus should have their HbA1C value checked every three months. The normal range is 4-6% of total hemoglobin. Diabetics should also try to keep their level below 8% to prevent permanent damage from the disease. In patients with iron deficiency anaemia, liver cirrhosis, renal insufficiency and other diseases that can influence the blood count, the values are falsified and not meaningful.