Clinical Characteristics of Hemophagocytic Syndrome: 46 Cases Report and Literature Re-View

Objective

HPS is caused by a variety of diseases, the most common causes including infections, malignancies and autoimmune diseases. It has an acute onset, a dangerous condition, rapid progress, and a high mortality rate. The main clinical manifestations are persistent fever, hepatosplenomegaly, hematocytopenia and tissue cell phagocytosis.Most of the current research is focused on children, and there are few reports on adult HPS.And lack of multi-center, prospective research, its pathogenesis, diagnosis, treatment is still poorly understood.

Methods

To analyze the clinical manifestations,laboratory data, therapy and prognosis in patients with hemophagocytic syndrome, 46 patients (over 18 years old) with HPS in our hospital from June 2011 to September 2017 were analyzed.The scope of the study included the patient's age, gender, etiology, clinical manifestations, laboratory indicators, treatment and prognosis, and analyzed its clinical, laboratory and prognostic characteristics.

Result

Of the 46 patient cases, 19 cases were secondary to cancer and 11 cases secondary to infection,10 cases were considered as malignant lymphoma by PET- CT,other 6 cases of unknown etiology.A total of 31 patients with EBV infection were diagnosed by EBV-DNA in all patients.Forty-six patients had various clinical manifestations, all had fever, mostly persistent irregular hyperthermia, and antibiotic treatment was not effective.There are also splenomegaly, serous effusion, respiratory symptoms, hemorrhage, central nervous system symptoms, renal dysfunction。According to HPS -2004 criteria,the coincidence rate of indices were: fever (100%), high level of serun ferritin (100% ),cytopenias (93.48% ),splenomegaly (91.30% ), hemophagocytosis in bone marrow,spleen or lymph nodes (84.78% ),hypofibrinogenemia (67.39% ), hypertriglyceridemia (54.05% ).

Among cancer,infection and unknown etiology group,there were statistically differences on Ferritin and β2 microglobulin (P<0.05), It can be seen that the levels of Ferritin and β2 microglobulin in the tumor-associated HPS group were significantly higher than those in the unknown etiology and infection-related HPS group.

The overall survival rate of the 46 patients was 11.1%.Causes of death included respiratory and circulatory failure (36.4%), cardiac arrest (18.2%), multiple organ failure (18.2%), hemorrhage, cerebral palsy (18.2%), and liver failure (9.1%).Comparing the laboratory indicators of the survival group and the death group at the initial diagnosis, the Lactic dehydrogenase and Triglyceride levels in the death group were higher than those in the survival group, while the Fibrinogen level in the death group was lower than that in the survival group.

Conclusion

The secondary HPS occurs from various underlined disease,many associated with Epstein- Barr virus infection.Cancer,especially NK/T- cell lymphoma is the main cause.Adult HPS progresses rapidly due to the disease, which makes it difficult to confirm the diagnosis in time. Some patients have deteriorated before the diagnosis is perfected.Increased sensitivity indicators such as sCD25 and NK cell activity can help early diagnosis.

The 1-month survival rate of the chemotherapy group was higher than that of the anti-infective group alone, indicating that chemotherapy and controlled hemorrhage are meaningful for controlling the disease in a short period of time, while the simple anti-infection effect is poor.Adult HPS has only gradually gained attention in recent years. How to identify the underlying cause of HPS and to formulate a treatment strategy based on different incentives and prognostic factors is essential.