[Clinical analysis of 13 patients with hemophagocytic syndrome].

OBJECTIVE

To investigate the clinical features and prognosis of hemophagocytic syndrome (HS).

METHODS

The clinical symptoms, signs, and laboratory-test data in 13 patients with HS were analysed.

RESULTS

Increase in lactate dehydrogenase (LDH) and hyponatremia was found in all of the patients. Prolonged prothrombin time, hypofibrinogenemia, hyertriglyceridemia, and hyperferricemia also existed in some cases. The mature hemophagocytic histocyte and hemophagocytic phenomenon were observed with Whrigt-Geimsa and immunocytochemical staining. One (16.6%, 1/6) patient with infectious associated HS (IAHS) and 4 (80%, 4/5) with non-IAHS died of infection and primary disease.

CONCLUSION

HS especially non-IAHS is an extremely dangerous state with high mortality. Obstinate hyponatremia may be a characteristic of HS in the early stage. It's important to supervise the change of hemophagocyte in peripheral blood and bone marrow of HS. Immunocytochemical studies on smear of enriched peripheral white blood cells are helpful to identify the primary pathogenesis of the benign or malignant diseases.