Hemophagocytic syndrome

When it comes to diseases like hemophagocytic syndrome, many friends do not understand what kind of disease it is. In fact, hemophagocytic syndrome is divided into two types: primary and secondary. The former is mainly related to autosomal genetic material, that is, congenital chromosomal abnormalities, while the latter is related to viral infections such as bacteria. So what are the symptoms of hemophagocytic syndrome?

In fact, the treatment of hemophagocytic syndrome is relatively complicated. In addition to timely surgical treatment, it also requires the use of a large number of drugs as treatment methods, etc. The following is a detailed introduction to the disease of hemophagocytic syndrome.

Hemophagocytic syndrome

Causes

Hemophagocytic syndrome is mainly divided into primary (hereditary) and secondary. The former is autosomal recessive or X-linked, with a clear gene defect or family history. The latter can be caused by a variety of factors, including infection (mainly Epstein-Barr virus infection), malignant tumors, autoimmune diseases, drugs, acquired immunodeficiency (such as transplantation), etc.

Clinical manifestations

1. Familial hemophagocytic syndrome: The age of onset is generally early, most occurring within 1 year old, but there are also older patients who develop the disease. Clinical manifestations vary. In the early stages, they are mostly fever, hepatosplenomegaly, and may also include rash, lymphadenopathy, and neurological symptoms. Fever is often persistent and may subside on its own. The liver and spleen are obviously enlarged.

2. Secondary hemophagocytic syndrome: (1) Infection-related hemophagocytic syndrome Severe infection can cause a strong immune response, which often occurs in immunocompromised patients. It is usually caused by a virus, but may also be caused by bacteria, fungi, rickettsial, and protozoan infections. (2) Tumor-associated hemophagocytic syndrome Acute leukemia, lymphoma, seminoma, etc. may be complicated by or secondary to hemophagocytic syndrome before, during, or after treatment. (3) Macrophage activation syndrome is a serious complication of chronic rheumatic diseases in children and is more common in patients with systemic juvenile rheumatoid arthritis. On the basis of chronic rheumatic diseases, patients develop symptoms of hemophagocytic syndrome such as fever, hepatosplenomegaly, pancytopenia, abnormal liver function and central nervous system lesions.

treat

The prognosis of familial hemophagocytic syndrome is poor and the disease progresses rapidly. Bone marrow transplantation is recommended as soon as possible. The treatment of secondary hemophagocytic syndrome is more complicated. On the one hand, treatment must be directed at the primary disease. For example, blood/lymphatic system tumors require chemotherapy, and infection-related hemophagocytic syndrome requires anti-infection treatment. The hemophagocytic syndrome treatment regimen should be used simultaneously with the treatment of the primary disease to control the progression of the disease. Currently, the HLH-2004 regimen is widely used internationally to treat secondary hemophagocytic syndrome. If treatment is refractory, fails, or the disease relapses, bone marrow transplantation may be considered.

Regarding hemophagocytic syndrome, experts told us that if it is a disease caused by family genetics, most patients will develop the disease within 1 year old, of course there are also older patients. The best treatment is bone marrow transplantation. Secondary diseases require treatment with certain medications in addition to chemotherapy.