What is macrophage activation syndrome (HLH)?

Macrophage activation syndrome is a rare but potentially life-threatening disorder. This syndrome is still very new in Vietnam and is not known to many people, so the patient's life is very likely to be in danger if not diagnosed and treated promptly.

Hemophagocytic Lymphohistiocytosis (HLH) is a rare blood disease with a mortality rate of 15-60%, the disease is common in children under 10 years of age. appears in children under 1 year of age while the secondary form does not appear until about 6 years of age. This is a syndrome where histocytes are overactive, leading to red blood cells and platelets. , white blood cells are phagocytosed.
This syndrome is divided into two types, primary and secondary
Primary form, also known as familial form: This is characterized by recessive inheritance, the causative gene is located on chromosomes 9 and 10. onset: The syndrome is formed after or during the patient's encounter with some condition such as viral infection, bacteria, parasite, blood cancer, lymphoma, lupus... Phagocytic cells are found. It is found in bone marrow and is derived from pluripotent cells. The exact process of macrophage is not known, when the body is infected with viruses or bacteria causing phagocytic cells to be activated.

2.1 Criteria according to the World Association for Hemophagocytosis Confirm the diagnosis through molecular genetic testing due to the presence of a mutation in one of the genes involved in this syndrome.
In 2004, the World Association of Hemophagophageal Pathology identified 5 diagnostic criteria for this syndrome including:
Fever Splenomegaly At least 2 decreased peripheral bloodlines Concurrently elevated blood triglycerides and fibrinogen or increased triglycerides and decreased fibrinogen. Perform myelogram, splenogram or lymphangiogram showing hematopoietic phagocytosis. By 2008, the Association had introduced 3 new diagnostic criteria including:
Decrease or loss of Ferritin NK cell activity ≥ 500 ng/ml soluble CD25 ≥ 2400U/mL. 2.2 For atypical forms For atypical forms of HLH, the diagnosis is based on the following factors:
Check for cloudy cerebrospinal fluid Liver biopsy shows chronic hepatitis Tests to rule out TB Some symptoms such as: Enlarged peripheral lymph nodes, jaundice, edema, skin rash, elevated liver enzymes, hypoproteinemia, hyponatremia If the patient does not meet the above diagnostic criteria but during the course of treatment If signs are confirmed, HLH should be treated immediately.

The ideal treatment for macrophage activation syndrome is to treat the underlying cause. However, because the disease progresses rapidly and can be life-threatening, the doctor will proceed to treat the symptoms that the patient is experiencing.
Drug therapy is the first treatment, commonly used drugs such as etoposide, dexamethasone, cyclosporine...
Current treatments for this syndrome include:
Treating the cause Using chemotherapy to prevent macrophage activation Plasma exchange Radiation therapy Marrow transplant this is a radical treatment for this syndrome If there is a need for consultation and examination at Vinmec Hospitals under the national health system, Please make an appointment on the website to be served.