Today it is The day of illness of the world CaslmanA field per day when doctors and patients take the opportunity to claim a greater study and awareness of this condition, known in the field of medicine as Imitating the diseaseFor his tendency to confuse with other disorders. Usually consists of A benign tumorWhose complications can make it more serious than some evil. Therefore, it is very important that your diagnosis is as early as possible. Unfortunately, being classified in the group Rare diseasesIt does not always attract the attention that should.
IN Hypertual We talked with NaomiOne of 100 people with Caslman’s disease, which is evaluated in Spain. We also interviewed the doctor Juan Vila AlvarezThe Director General of Recuri is rare diseases in Spain, Portugal and Turkic. Both made us understand this rare disease and the reason why it is so important to increase awareness of this.
This is a disease that requires more research and more training among toilets so that diagnostics can be much faster and accurate. This is important for improving the quality of life of patients, as well as reduce the risk of death in the most serious cases. As a rule, this is not a deadly disease, but in some cases it can cause fatal organic failures. Time, as in many other rare diseases, is gold.
What is currently known about Kaslman’s disease?
Like cancer, in fact, Kaslman’s disease is not the only disease, but a very wide group of them. In general, increased lymph nodes are involved; That is, lymph nodes that expand and cause a wide range of symptoms.
The most common form of disorder implies one enlarged lymph node, usually located In the chest or neckAlthough it can also be found in other areas of the body. This is what is known as The disease of the unanocentric Caslman.
In the event that there are slightly increased nodes, we will talk about Multicentric disease of KaslmanIt causes inflammatory symptoms and problems with the functionality of the organs and, in turn, covers three more types of disease: Caslman’s multicentric disease associated with VHH-8, idiopathic or related to verses.
The first, as its name indicates, is produced Human virus herpes 8, Although HIV. In the second, the origin is unknown. It is known that this is not connected with the herpes virus 8. Its severity can be very variable. Finally, the latter is associated with another attachment called Polyneuropathy syndrome, visceromegaly, endocrinopathy, m -bead and skin changes. This is a rare blood disorder that damages the nerves and affects other parts of the body.
What are usually the first alarm signals?
According to Dr. Vila Alvareza, There are some forms of disease that is asymptomatic While in other cases, patients have more symptoms. “Some symptoms that can cause a suspicion of this disease: Inflamed lymph nodes, fever, night sweating, intensive fatigue, weight loss, anemia and increasing inflammatory markers. ”
For example, in the case of Noemí, he tells us that the first symptoms that forced him to go to the doctor were Extreme pain in the joints, fatigue and asymptomatic fever.
All these are symptoms that can also occur in other diseases. Even being what can be called a benign tumor, it can be confused with cancer, as well as with autoimmune diseases, such as lupus or infections, such as mononucleosis. This is just the biggest problem of the disease, as the doctor reminds us. “If the clinician does not suspect the disease in relation to these symptoms, it is likely that the patient Do not face diagnosis“
In soft cases, this does not imply a big problem. However, in the most serious cases, the late diagnosis can be fatal.
What will happen if it is not diagnosed?
“A delay in the diagnosis may have consequences for the health and life of patients,” says Dr. Vila Alvarez. “If they do not receive proper treatment in time, the disease will continue its course, the ability to influence Other organs“In the most serious cases” this will increase the opportunity to suffer from complications, such as Insufficiency of organs, serious infections, or even obtaining in very weakening or mortal situations“
To make this necessary diagnosis, it is necessary to combine three types of tests. On the one side, Blood and urine testsIn which inflammation markers and blood cells are analyzed, and, of course, other diseases are discarded. Also necessary Images tests, with which the expansion of the nodes is analyzed. This is very useful, for example, Computed tomographyField and, finally, it is necessary Ganglia biopsyThe field also helps to see if there is a benign tumor and exclude other possible conditions.
This is a process that does not have to be long, but this is often postponed only because of the ignorance that exists in relation to this disease. For example, in the case of noemí, he was supposed to Go a long way Until you make your diagnosis. “Since I visited the hospital of Ramon and Kajal, the diagnosis took only a month, but until it passes at least three years of specialist and from one hospital to another without results.”
How is Caslman’s disease?
As soon as the disease is diagnosed, it is important to look for treatment as soon as possible. This will depend on the type and severity of Kaslman’s disease, which was diagnosed, as Dr. Vila Alvarez tells us. “In one case, The inflammatory ganglion is removed And this operation is performed with a healing intention, ”he says.” On the contrary, a more complex approach is necessary in a multicenter form, and between the options and between the options Immunotherapy, monoclonal antibodies, corticosteroids or even chemotherapy”
For example, Noemí tells us that she currently takes SiltuximabMonoclonal antibody, widely used to treat Caslman’s disease. Nevertheless, they continue to study their symptoms in order to clarify more treatment, since they are very diverse.
It should be noted that the most serious ways need to be treated Long or vague Control the disease and avoid new relapses.
The latter is vital to avoid complications. And in the most serious cases, while 35% of patients They could die for five years after the diagnosis, if they do not receive proper treatment.
Why is it important to talk about this and other rare diseases?
Rare diseases suffer from serious Lack of researchThe field but this is not all, among the population there is not enough knowledge and much less. If medical workers themselves do not have knowledge and instruments necessary for diagnosis, patients can have a much worst quality of life.
In case of Caslman’s illness, Dr. Vila Alvarez is clear. “Only an improvement in knowledge about this pathology will be achieved that clinicians can more easily recognize it by reducing time to the diagnosis of patients,” he explains. “From a reminder, in our desire to find solutions for minority diseases, we facilitate continuous medical training and provide medical workers with the opportunity to get exchange the latest data and scientific knowledge In pathologies such as Kaslman’s disease. “
Noemí, on the other hand, believes that it is also Social problem. “If doctors do not know them, they cannot diagnose it, but social knowledge is also important for the formation of a support network around the patient.”
It is very sad that rare diseases are synonymous with unknown or even unattended diseases. That is why it is so important that there are researchers who devote their lives in order to understand them better and the network of patients fighting for the publication of what is happening to them. That is why it is also necessary that there is such a day. Although Kaslman’s disease, like the rest of the rare diseases, should speak every day of the year.
Source: Hiper Textual
