Hello! I have been a doctor for 21 years. My name is Georgy Olegovich Sapego. In this article I will talk about hemophagocytic syndrome.
This story will have eaters, killers, cytokine storms, triggers and unrealistically high ferritin.
Hemo means blood. And phagocytes are cells that can swallow other cells. Therefore, hemophagocytic syndrome is a disease in which individual cells of our immunity begin to swallow blood cells. They are literally being devoured. This is not jargon.
Eaters
Macrophages are involved in such an outrage. These are immune cells. They look like amoebas. They swim up to other cells or microbes, like amoebas envelop them with their processes, pull them in and digest them. In the past, macrophages were called eater macrophages. Quite an official name.
Hemophagocytic syndrome is more common in infants, but adults also get sick. This is a dangerous disease that is found late because it is very vague.
The disease is relatively new. She was found in 1952. Hemophagocytic syndrome is usually triggered by some kind of infection, but hereditary predisposition is of great importance.
If macrophages begin to devour blood cells, then anemia, bleeding, and various organs will suffer.
Killers
In addition to macrophages, a variety of lymphocytes, which are called natural killers, are also involved in this disgrace. Again, this is not jargon, but the official name. These lymphocytes specialize in killing their own cells. If viruses attacked our cells, or the cells turned into tumor cells, then they will only harm, and such cells will have to be killed. For this, our body has the very same killer lymphocytes.
Cytokine storm
Immunity and inflammation in our body are regulated by special signaling substances. They are called cytokines. In yesterday's article about anemia of chronic diseases there was just a point about cytokines. If there are too many such substances, then they can start a real war inside our body. This is called a cytokine storm.
Triggers
A trigger is a trigger. Something that can trigger a powerful reaction in our body. We have already talked about triggers in the topic of bronchial asthma. Allergens can be triggers there. In the story of hemophagocytic syndrome, the trigger is usually some kind of infection.
As it will be
It will look like an infection at first. The body temperature will rise, the spleen will increase, the level of erythrocytes and platelets in the blood will begin to decrease. Another kind of anemia will turn out.
Ferritin
Ferritin stores iron in our body. If there is not enough ferritin, then this means that we lack iron. The story about the lack of iron is very fashionable. Ferritin is now being tested at every turn.
Doctors are constantly debating what is normal ferritin levels. Someone says that ferritin should not be higher than 80 ng / ml, someone like the American standards, where the upper limit of ferritin is about 200 ng / ml. And with hemophagocytic syndrome, ferritin can increase to 500, 5000 and even 10,000 (ten thousand!) Ng / ml.
This is so common in hemophagocytic syndrome that a ferritin level of more than 2000 ng / ml is almost certainly indicative of it. The higher the ferritin level, the worse it all ends.
Hematologists are involved in the treatment of hemophagocytic syndrome. It's terribly difficult. The worst part is that this thing is found late. A person can be played in hospitals for several months and treated with varying degrees of success. From which the conclusion - do not run between doctors in different medical institutions, but bring your examination to its logical conclusion.
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