How to relieve blood syndrome?

People are not so clear about blood syndrome. This is mainly because the occurrence of this disease is still very rare and the incidence rate is not that high. Most patients have symptoms of this disease at birth. So far, the treatment effect of this disease is not so good, and there is not even a method that can be used to completely cure it. Patients will be troubled by the disease for a long time and cannot get better relief. Now they can only temporarily relieve the symptoms through bone marrow transplantation.

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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. The 2004 regimen was based on dexamethasone, etoposide and cyclosporine, and was divided into an initial treatment period of 8 weeks and a maintenance treatment period, with an additional intrathecal injection. The use of immunoglobulin in the acute phase can help relieve the condition.

If treatment is refractory, fails, or the disease relapses, bone marrow transplantation may be considered.

First of all, it is necessary to distinguish whether it is congenital or secondary, especially to distinguish the former from virus-related hemophagocytic syndrome. Secondly, tumor-associated hemophagocytic syndrome must be strictly excluded. Since this disease is staged, early patients may not show all the symptoms, making early diagnosis difficult. Repeated bone marrow examinations or related examinations may help with early diagnosis.

Hemophagocytic syndrome (HLH) is considered to be a histiocytic disease with reactive proliferation of the mononuclear macrophage system. It is mainly caused by defects in the function of cytotoxic killer cells (CTL) and NK cells, which lead to antigen clearance disorders, excessive activation and proliferation of the mononuclear macrophage system due to continuous antigen stimulation, and the production of a large number of inflammatory cytokines, resulting in a group of clinical syndromes. The main manifestations of hemophagocytic syndrome are fever, splenomegaly, pancytopenia, high triglycerides, low fibrinogen, high serum ferritin, and hemophagocytosis may be found in bone marrow, spleen or lymph node biopsy.