How long can a person with hemophagocytic syndrome live?

Hemophagocytic syndrome is a non-contagious disease. Clinically, the disease can be primary or reactive. It may be caused by infections, tumors and other diseases. It usually occurs in elderly people with weak constitutions and people with poor immunity. Experts say that if the infection factors can be contained in time, there is a possibility of self-healing. If it is reactive, it will take some time and will not endanger life safety.

1. Introduction to Hemophagocytic Syndrome

Hemophagocytic syndrome can be divided into primary and reactive types. The underlying diseases may be infection, tumor, immune-mediated diseases, etc. Due to the increase in hemophagocytic cells, the destruction of blood cells is accelerated. Because the elderly have weaker constitutions and poorer immunity, they are more likely to get infected. This disease is generally not contagious. If it is an infectious factor, the infectious factor must be removed as soon as possible. The primary disease can usually heal itself after treatment.

2. Diagnostic criteria for hemophagocytic syndrome

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.