Hemophagocytic syndrome

The causes and symptoms of hemophagocytic syndrome

Hemophagocytic syndrome and its causes

Over the past decade, considerable success has been achieved in the study of biological features of the system of macrophages, which is considered as a biological filter the blood and lymph circulation, promotes the removal of microorganisms, cells affected tumor and infected viruses, toxins, metabolites, residues of drugs.

In the process of cleansing the body involves mononuclear phagocytes of the liver and spleen, monocytes, macrophages of lymph nodes, alveolar macrophages.

Increased activity of macrophages leads to non-regulated phagocytosis of red blood cells - this is a distinctive feature of hemophagocytic syndrome (HPS). The disease is hereditary or acquired, belongs to the group gistiotitarnaya syndromes combining disease characterized by the proliferation of monocytes, macrophages and dendritic cells.

Symptoms of GFS

The symptoms described in 1952. stable is characterized by fever, reduction of substances form the blood, enlarged liver and spleen with signs of pronounced hemorrhagic syndrome.

In patients with marked disruption of the liver, elevated levels of ferritin and transaminases, neurological symptoms with malfunction of the Central nervous system, elevated levels of triglycerides in plasma, coagulopathy with increased blood clotting.

The clinical picture of many patients, enlarged lymph nodes, rashes, jaundice and swelling. In the spleen red pulp, liver sinusoids, lymph nodes sinuses, in the bone marrow and the Central nervous system in the manifestations of HFS there is diffuse infiltration of organs and tissues activated macrophages with signs of hemophagocytosis. When this is depleted lymphoid tissue. Liver biopsy shows changes typical of chronic persistent hepatitis.

There are two different States, initial clinical symptoms are difficult to distinguish from each other.

1. Hereditary hemophagocytic lymphohistiocytosis is an autosomal recessive disorder in which mutation in the gene for perforin plays an important role (20-40% of cases SGLG).
2. Lymphohistiocytosis secondary hemophagocytic syndrome, macrophage activation hemophagocytosis that develops in the result of immune activation of mononuclear phagocyte system. In most cases, patients with hemophagocyticlymphohistiocytosis there is no immunodeficiency. The development of HLH can be induced by rheumatic diseases, malignant tumors, metabolic diseases.

Forms of hemophagocytic syndrome

To date, there are four forms of the disease, three of which identified the genes responsible for the synthesis of proteins. They are involved in mediating CD8 T and NK cell cytotoxicity and encode perforin – a protein embedded in a lipid bilayer membrane of a target cell and triggering its death.

Diagnosis of hemophagocytic syndrome

Incredibly difficult the differential diagnosis between primary and secondary HLH in the absence of family history. For diagnosis requires histological identification of hemophagocytosis in the organs. Identified pathology is difficult to assess on the basis of biopsies of bone marrow, lymph nodes or liver. Immunologic tests showing the inhibition of the activity of NK cells and an increase in the level of the receptor of interleukin-2 are not decisive in the diagnosis. The diagnosis of HLH is established on the basis of symptom complex that included hepatomegaly, splenomegaly, leukopenia, thrombocytopenia, anemia, hypofibrinogenemia, hypertriglyceridemia, CNS. Final verification of the form of the disease is based on molecular genetic analysis. Probably the most common in adults SFS SFS found in the form of IGFS. Viral infection, especially caused by Epstein – Barr (EBV), can initiate both primary and secondary HLH.

Treatment of hemophagocytic syndrome

Treatment should begin at the early stages of the disease. Early treatment with immunosuppressants, the use of antiviral drugs, plasmapheresis and symptomatic therapy will help to hemophagocytic syndrome was not recurrent, severe and persistent with irreversible consequences.

The treatment Protocol used, chemotherapy, allogeneic transplantation of hematopoietic stem cells from a compatible donor, it contributes to the gradual recovery. Unfortunately, this disease is very insidious, often conducted by splenectomy promotes recovery and well-being of patients, allowing you to live a normal life and work.