If it’s severe, you lose your life within a week “this disease” … Suspected symptoms?
Understanding and Health Shipbuilding Reporter
2022/02/08 20:30
Mild symptoms such as lethargy, skin rash, and edema are also at risk
▲ Hematopoietic lymphohistocytosis can be misunderstood as a simple body in the early days./ Photo=Clipart Korea
There is a rare disease with a very low 5-year survival rate of 55% of patients. It is’hemopoietic lymphohistocytosis’. The disease, which is unfamiliar in name, is one of the immunodeficiency diseases, with a high lethality, and if it is not detected early, the disease progresses rapidly and in severe cases loses its life within a week to two months. However, most often the treatment time is missed because of the sudden progression of symptoms that do not interfere with life. Hematopoietic lymphohistocytosis is a disease that is especially difficult to diagnose and has a poor prognosis, so it is necessary to know exactly and be careful.
◇Symptoms such as fever, hepatomegaly, weight loss, etc.
Hematopoietic lymphohistocytosis is a kind of rare incurable disease that occurs while eating normal hematopoietic cells such as red blood cells and white blood cells are abnormally activated macrophages and lymphocytes that prey on bacteria and viruses. That is, abnormal symptoms appear as cytokines that are important for the production and maintenance of immune responses are over-secreted. It is divided into primary and secondary by heredity. Genetic problems occur mainly in children. On the other hand, secondary can occur at any age because it can occur from a variety of causes, including infections, tumors, etc.
Primary hemopoietic lymphohistocytosis arising from genetic problems is also called hereditary or familial hemopoietic lymphohistocytosis can occur due to variations in various genes. Secondary hemopoietic lymphohistocytosis is a powerful immunological activation caused secondarily by various causes, cytokine storms are the cause. It is most often associated with severe viral infections or malignant tumors. The reported sources of infection are very diverse, and typical viruses include adenovirus, cytomegalovirus, Epstein-barr virus, human immunodeficiency virus, etc. Other bacteria, fungi, mycobacterium tuberculosis, parasites, etc.are included. It may be accompanied by rheumatic disease or other autoimmune diseases.
The time of manifestation of the symptoms of hemopoietic lymphohistocytosis is not clear, but depending on the cause, several symptoms may appear simultaneously. First, typical symptoms include fever, hepatosplenomegaly, hemopenia, weight loss, etc.following upper airway(pharynx, larynx, nasal cavity) infection or gastrointestinal tract infection. In addition, skin rashes, jaundice and edema, lymph node hypertrophy or pain may appear. In the early days, nonspecific symptoms such as mild fever and lymph node hypertrophy may appear, so you can think of a simple body, but according to the abnormal immune response of the individual, coagulation disorders, neurological symptoms, multiple organ failure, etc., fatal disease exacerbation occurs suddenly, so it is very difficult to predict.
◇Help with sIL2R diagnosis, disease identification and prognosis prediction
Hematopoietic lymphohistocytosis, also known as cytokine storm, can be diagnosed through various tests. Genetic tests and various tests are performed at the same time, along with clinical aspects such as fever, splenomegaly, etc., and the results of the tests are determined comprehensively and used for diagnosis. Total blood count(Complete blood count), neutral fat in the fasting state, fiber source, ferritin, natural killer cell activity(Natural Killer cell activity), sIL2R (Soluble IL-2 receptor, interleukin-soluble receptor or Soluble CD25)includes blood tests such as tests, other bone marrow tests and lymph nodes, liver and other organs suspected of invasion are performed to check for hemoptysis or viral infections or tumors.
Of these, sIL2R is the only cytokine receptor included in the diagnostic criteria for hemophagocytic lymphohistocytosis among various cytokines or cytokine receptors. It is present at low levels in the serum of healthy people, but at high concentrations in cytokine hypersecretion diseases and conditions in which T lymphocytes are activated, such as tumors, autoimmune diseases, infectious diseases, etc. If the sIL2R concentration is higher than 2400U / mL, it meets one of the eight diagnostic categories for the diagnosis of hemocytopenic lymphohistocytosis, so it is useful for diagnosis and prognosis prediction.
If hemophagocytic lymphohistocytosis has been diagnosed, rapid treatment should be carried out before the disease worsens. It is necessary to suppress life-threatening severe hyperinflammatory conditions and treat comorbid diseases and conditions that cause them. In hereditary cases, hematopoietic cell transplantation therapy is necessary to ultimately restore the defective immune system. If the antibiotic treatment is a possible infection is the cause, it is also treated while administering the appropriate antibiotics to treat the source of the infection, depending on the patient’s symptoms and condition, anti-cancer drugs and immunosuppressant injections may be used.
The cause of hematopoietic lymphohistocytosis has not yet been clearly identified, and although diagnosis and treatment are difficult, regarding half of patients are known to have succeeded in treatment. However, if the diagnosis is delayed, it may be unfortunate that the disease has already worsened and does not see the effect of treatment.
Choi Li-hwa, a specialist at GC Green Cross Medical Foundation, said, “If hemophagocytic lymphohistocytosis develops, the probability of death in the short term is very high if it is not found early because cytokine storms due to excess of the immune response may be the cause,”and said, “If you suspect the disease, various blood tests such as sIL2R test used to diagnose hemophagocytic lymphohistocytosis can help
