[Seven cases of hemophagocytic syndrome complicated with childhood collagen diseases].

We encountered seven children with hemophagocytic syndrome complicated with collagen diseases of whom five were JRA and two SLE, HPS was seen as one of initial symptoms in 6 cases excluding one case of JRA. Hematologically, cytopenia in 2 cell lines (RBC and platelets) was seen in most cases. Active hemophagocytosis by matured large histiocytes was observed in all cases. All patients had moderately to extremely high serum ferritin levels and LDH levels. Although both lives did not correlated with prognosis in HPS with collagen disease, the higher serum total cholesterol/serum triglyceride ratio, the worse was the prognosis. Those findings suggested that HPS in collagen diseases may be a different entity from other types of HPS.