Hemophagocytic syndrome

Introduction The phagocytic cells in the human blood are a double-edged sword. On the one hand, like a cleaner, they invade the invading bacteria and the aging cells of the body, clean up the harmful substances and protect the human body. On the other hand, if they are excessive If you are active, you will be able to devour the useful cells of the human body. It will seriously erode human tissues and organs, causing a series of injuries, leading to serious consequences such as major bleeding, liver failure and respiratory failure. This is the hemophagocytic syndrome.

Cause

Pathogenesis:

Hematopoietic cell syndrome can be divided into primary occurrence and responsiveness. Potential diseases can be infections, tumors, immune-mediated diseases, etc. Due to the increase of hemophagocytic cells, the destruction of blood cells is accelerated. Because the physical condition of the elderly is weak and the immunity is poor, it is more likely to cause infection. This disease is generally not contagious. If it is an infection factor, the infection factor should be removed as soon as possible. After the primary disease is treated, it can be self-healing.

Pathological features:

Benign tissue cells increase with bloodthirsty, more common in lymph nodes of the lymphatic sinus and myelin, hepatic sinus, portal vein, red pulp and bone marrow of the spleen. In the acute phase, the disease is similar to leukemia, malignant histiocytosis, infectious mononucleosis, etc., and not all cases can be found in the first bone penetration, and sometimes need multiple punctures to confirm the diagnosis. The bone marrow is hyperproliferative, the proportion of the granulocyte system is reduced, and the neutrophils are toxic. The proliferation of the young red system was normal, and the proportion of the lymphatic system was not significantly changed. Mononuclear macrophage system is active, often >10%, macrophage size is 20-40 microns, or larger, cytoplasm is abundant, phagocytosis of multiple mature red blood cells, or young red blood cells or platelets. Megakaryocytes are roughly normal.

an examination

Related inspection

Blood routine lumbar puncture blood test

Blood routine: blood cell reduction (peripheral blood or blood tridymia), wherein hemoglobin <90g / L, platelets <100 x 109 / L, neutrophils <1.Ox109 / L;

Blood test: hypertriglyceridemia and / or hypofibrinogenemia;

Lumbar puncture: Bone marrow, spleen or lymph nodes showed hemophagocytic cells but no malignant manifestations.

Differential diagnosis

Diagnostic criteria:

1, fever: fever more than 1 week, heat peak > 38.5'C

2, hepatosplenomegaly: liver and spleen with complete blood cell reduction, cumulative >= 2 cell lines

3, blood cell reduction (peripheral blood or blood triad cells decreased), including hemoglobin <90g / L, platelets <100 x 109 / L, neutrophils <1.Ox109 / L

4. Hypertriglyceridemia and/or hypofibrinogenemia

5, bone marrow, spleen or lymph nodes can be seen in blood cells but no malignant manifestations.

Infectious hemophagocytic syndrome is a benign hemophagocytic hyperplasia associated with acute viral infection, which occurs mostly in children. It is characterized by mononuclear-macrophage hyperplasia and obvious phagocytosis of red blood cells. Patients have obvious high fever, liver, spleen and lymph nodes, and the primary disease can be self-healing after treatment. The patient has anemia, and the white blood cells are significantly reduced. The lymphocytes are obviously increased in the classification, and the lymphocytes are easy to be seen. Platelets are often reduced.

The bone marrow is hyperproliferative, the proportion of the granulocyte system is reduced, and the neutrophils are toxic. The proliferation of the young red system was normal, and the proportion of the lymphatic system was not significantly changed. Mononuclear macrophage system is active, often >10%, macrophage size is 20-40 microns, or larger, cytoplasm is abundant, phagocytosis of multiple mature red blood cells, or young red blood cells or platelets. Megakaryocytes are roughly normal.

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