What are the diagnostic criteria for hemophagocytic syndrome

Most people are not very clear about the disease called hemophagocytic syndrome, or have never heard of it. In fact, this disease will directly endanger the patient's life after it occurs, causing the patient to remain in a state of high fever with no way to reduce the fever in a short period of time. If a blood test is conducted, it will be found that the blood cells in the body are gradually decreasing, which will make the coagulation function become worse and worse, resulting in more serious consequences.

Diagnostic criteria

1. Fever: Fever for more than 1 week, peak temperature > 38.5'C

2. Hepatosplenomegaly: Hepatosplenomegaly with pancytopenia, cumulatively >= 2 cell lines

3. Cytopenia (decreased peripheral blood cells of the secondary or tertiary lines) , with hemoglobin <90 g/L, platelets <100 x 109/L, and neutrophils <1.0 x 109/L

4. Hypertriglyceridemia and/or hypofibrinogenemia

5. Hemophagocytic cells may be seen in the bone marrow, spleen or lymph nodes but without malignant manifestations .

Infectious hemophagocytic syndrome is a benign hemophagocytic histiocytosis associated with acute viral infection. It often occurs in children and is characterized by active proliferation of monocytes and macrophages and obvious phagocytosis of red blood cells. Most patients have obvious high fever, enlarged liver, spleen and lymph nodes, and most of them can recover on their own after the primary disease is treated. The patient has anemia, a significant decrease in white blood cells, and classification shows a significant increase in lymphocytes, which is prone to abnormal lymphocytes. Platelets are often decreased.

The bone marrow is actively proliferating, the proportion of the granulocyte system is reduced, and neutrophils may become toxic. The proliferation of the erythroid system is mostly normal, and the proportion of the lymphatic system has not changed significantly, but abnormal lymphoma may be seen. The mononuclear macrophage system proliferates actively, often >10%. The macrophages are 20~40 microns in size, or larger, with rich cytoplasm, and can phagocytose multiple mature red blood cells, or immature red blood cells or platelets. Megakaryocytes are generally normal.