What are the symptoms of hemophagocytic syndrome

Hemophagocytic syndrome is an uncommon disease, but it can be fatal. If you do not take the disease seriously and receive timely treatment, the risk of death from the disease is extremely high. Hemophagocytic syndrome presents many symptoms, but none of them have any specific characteristics, so it is easy to misdiagnose or miss the diagnosis during examination. In general, patients will have fever and enlarged liver and spleen. Many people with poor physical constitution and low immunity are prone to this disease. Now let’s talk about the symptoms of hemophilic syndrome.

What are the symptoms of hemophagocytic syndrome?

1. Symptom diversity

The symptoms of hemophagocytic syndrome are characterized by many aspects, but most of them are atypical. Abnormal clinical symptoms usually include fever and splenomegaly. In addition, there are abnormalities in laboratory test data such as pancytopenia, high triglycerides, low fibrinogen, and high serum ferritin. In addition, hemophagocytic syndrome may be found in bone marrow, spleen or lymph node biopsy.

2. Diagnostic criteria

Fever lasts for more than 1 week, with peak temperature >38.5°C. Hepatosplenomegaly with pancytopenia, cumulatively >= 2 cell lines. Cytopenia (decreased peripheral blood cells of di- or tri-lineages), with hemoglobin <90 g/L, platelets <100 x 109/L, neutrophils <1.0 x 109/L, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytic cells can be seen in the bone marrow, spleen or lymph nodes but without malignant manifestations.

Treatment of hemophagocytic syndrome

1. Treatment of familial hemophagocytic syndrome

Familial hemophagocytic syndrome is a common type of hemophagocytic syndrome, which is caused by a certain genetic factor. Patients with this type have a poor prognosis and the disease progresses rapidly, so bone marrow transplantation must be performed as soon as possible. For patients with obvious physical symptoms, symptomatic treatment should be taken in a timely manner, mainly to improve and relieve physical symptoms and control the progression of the disease.

2. Treatment of secondary hemophagocytic syndrome

Unlike familial hemophagocytic syndrome, the treatment of secondary hemophagocytic syndrome is more complicated. The choice of treatment method must actively target the primary disease. For example, blood/lymphatic system tumors require chemotherapy, and infection-related hemophagocytic syndrome requires anti-infection treatment. At the same time, the hemophagocytic syndrome treatment plan should be used to control the progression of the disease. If treatment is refractory, fails, or the disease relapses, a bone marrow transplant may be considered.