Analysis of the clinical characteristics of reactive hemophagocytic syndrome

ObjectiveTo analyze the clinical presentation, laboratory data, treatment and prognosis of reactive hyemophagocytic syndrome(RHS).

MethodsThe clinical presentation, laboratory test, treatment protocols in 22 cases with RHS were retrospectively analyzed.The patients were divided into two groups according to the bone marrow hemocytology, and the difference between two groups was compared.

ResultsAmong 22 patients, the incidence rates of hyperpyrexia, lymphadenectasis, blood system involovement rate, anemia and thrombocytopenia were 95.5%, 81.8%, 72.7% and 50.0%, respectively.The levels of WBC, HB, ALB, TG and fibrinogen(Fbg) in RHS patients were(19.5±8.1)×10⁹/L, (95.2±14.4)g/L, (26.1±4.4)g/L, (3.2±2.1)mmol/L and(1.5 ±0.7)g/L, respectively.The bone marrow cytology Results showed the hemophagocytic phenomenon in 11 cases were indentified, and the difference of the positive indicators of examination between two groups were not statistically significant(P>0.05).The 50% patients alleviated after treatment, and discharged.Ten cases(one case lose to follow-up) died, which included adult Still disease in 5 cases, lymphoma in 1 case, dermatomyositis in 1 case, vasculitis related to anti-neutrophil cytoplasmic antibody in 1 case, systemic lupus erythematosus in 1 case and mesenteric lipid membrane inflammation in 1 case, and the respiratory failure was the death cause.

ConclusionsThe adult still disease in RHS caused by rheumatic disease is often found, the lymphoma is more, poor prognosis and high death rate, and the main death cause of which is respiratory failure.The related indicator test can help to early diagnose the RHS.Hormones combined with cyclophosphamide can control the progression of disease.