eosinophilic syndrome
Introduction
Introduction Hemophagocytic syndrome (HPS), also known as hemophagocytic cell hyperplasia, also known as hemophagocytic reticulosis, was first reported by Risdall et al in 1979. It is a multi-organ, multi-system involving, and progressively aggravated macrophage proliferative disease with immune disorders, representing a group of diseases with different pathogens, characterized by fever, hepatosplenomegaly, and complete blood cell reduction.
Cause
Cause
It is currently believed that there are many factors involved in the blood cell reduction of HPS patients: 1 hemophagocytosis, accelerate the destruction of blood cells; 2 inhibitory substances in the proliferation of hematopoietic progenitor cells in the serum, bone marrow granulocyte and erythroid precursor cells and megakaryocytes progressive The decrease is due to the production of inhibitory mononuclear factors and lymphokines such as gamma-interferon, tumor necrosis factor (TNF) and interleukin-1, and the production of hematopoietic growth inhibitory factor.
Examine
an examination
Related inspection
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Diagnostic criteria:
1 fever for more than 1 week, peak 38.5 ° C;
2 hepatosplenomegaly with complete blood cell reduction (involving 2 cell lines, bone marrow without hyperplasia or hyperplasia);
3 abnormal liver function (blood LDH normal mean + 3SD, generally 1000U / L) and coagulopathy (fibrinogen 1.5g / L), with ferritinemia ( normal mean + 3SD, generally 1000ng /ml);
4 hemophagocytic cells account for 3% of bone marrow smear nucleated cells, or histological manifestations involving bone marrow, lymph nodes, liver, spleen and central nervous system.
Diagnosis
Differential diagnosis
Differential diagnosis of red blood cell syndrome:
The most confusing is the identification of familial HPS and secondary HPS, especially with virus-associated HPS, because viral infections are not only associated with virus-associated HPS, but also in familial HPS patients, and familial HPS is also often induced by viral infections. Familial HPS is an autosomal recessive disorder, often without a family history, which increases the difficulty of diagnosis. It is generally believed that those who are ill before the age of 2 are more likely to be familial HPS, while those who are ill after the age of 8 are considered to be secondary HPS. In the case of 2 to 8 years old, it is judged according to clinical manifestations. If it is still difficult to be sure, it should be treated according to familial HPS. Secondly, it should be differentiated from malignant histiocytosis (malignant group), which is difficult to identify on bone marrow tablets, but HPS is much more common than the evil group. However, if clinical manifestations of outbreaks, severe liver damage, and high degree of malignancy in the bone marrow, especially in the liver, spleen or other organs, abnormal tissue infiltration is considered, it is appropriate to consider the evil group; otherwise, it should be diagnosed as HPS.
Diagnostic criteria:
1 fever for more than 1 week, peak 38.5 ° C;
2 hepatosplenomegaly with complete blood cell reduction (involving 2 cell lines, bone marrow without hyperplasia or hyperplasia);
3 abnormal liver function (blood LDH normal mean + 3SD, generally 1000U / L) and coagulopathy (fibrinogen 1.5g / L), with ferritinemia ( normal mean + 3SD, generally 1000ng /ml);
4 hemophagocytic cells account for 3% of bone marrow smear nucleated cells, or histological manifestations involving bone marrow, lymph nodes, liver, spleen and central nervous system.
