Hemophagocytic syndrome is a relatively common cell proliferative disease, the main cause of which is viral infection. Symptoms of hemophagocytic syndrome include fever, hepatosplenomegaly, hyperferritinemia, etc. If the body continues to have a fever, it is necessary to carry out corresponding inspections in a timely manner, because such continuous fever will affect the body's internal environment regulation. 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 . 6. Reduced or lack of NK cell activity 7. Hyperferritinemia 8. Elevated levels of soluble interleukin-2 receptor (sIL-2R)
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 can 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. |
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