Is sickle curable

Disease picture: what is sickle cell disease?

Author: PD Dr. med. Gesche Tallen, created on: 08/01/2011, editing: Maria Yiallouros, approval: Stephan Lobitz, Dr. med. R. Dickerhoff, Prof. Dr. med. U. Creutzig, last changed: 03.08.2012

Sickle cell disease is an inherited disease of the red blood cells (erythrocytes) that can affect the whole body. Erythrocytes are made in the bone marrow. As soon as they are ripe, they get into the blood. There they have the task, among other things, of transporting vital oxygen from the lungs to all other parts of the body. The oxygen is bound to the red blood pigment, hemoglobin. Normal hemoglobin consists of two different protein chains, which are known as alpha and beta chains. The way in which these chains assemble determines the spatial structure of the hemoglobin and is responsible for ensuring that the molecule can work properly.

Healthy red blood cells contain normal hemoglobin. This makes them round (see picture, black arrow) and supple, so that they can easily fit through even the smallest blood vessels and accordingly reach all organs in order to carry out their vital tasks. In sickle cell disease, the beta chain in hemoglobin is abnormally changed. In this case one speaks of "sickle cell hemoglobin". When sickle cell hemoglobin is not loaded with oxygen, it changes shape and, as a result, the shape of the red blood cells changes too. They are reminiscent of sickles, which gave the disease its name (see figure on the right, red arrow).

Sickle cells are more immobile and die earlier than healthy red blood cells (hemolysis). This leads to anemia on the one hand and clogging of the blood vessels by the sickle cells on the other. This means that the affected body regions do not receive sufficient oxygen. In turn, a lack of oxygen results in tissue damage, and when tissue is damaged, our body produces substances that cause pain. Sickle cell disease typically involves phases of severe pain (pain crises) and organ damage, particularly due to clogged blood vessels in the bone marrow, lungs, central nervous system, spleen and gastrointestinal tract. The spleen, which is damaged by blocked blood vessels in the first few years of life, is the cause of the high risk of life-threatening infections in sickle cell patients.

General information about bone marrow and blood

Information on the normal functioning of blood cells can be found in our text "Structure and function of bone marrow and blood" here

Normal red blood cells

Smear with normal red blood cells (erythrocytes) (© KPOH)

Sickle cells

Red blood cell smear from a patient with sickle cell disease. Black arrow: healthy red blood cells, red arrow: sickle cell (© KPOH)

COVID19 / sickle cell disease

The GPOH Consortium Sickle Cell Disease has published information on the risk to patients with sickle cell disease. Information for patients and doctors