EPIDEMIC MENINGOCOCCEMIA AND PURPURA FULMINANS WITH INDUCED PROTEIN-CDEFICIENCY

Patients with epidemic infections caused by Neisseria meningitidis ser ogroup C were studied to assess the relationship of abnormal coagulati on parameters to prognosis. Patients were categorized into stages with in the first hour of observation according to severity of illness. Dur ing the epidemic years 1986 through 1991, 113 patients with bacteriolo gically proven N. meningitidis infection were observed, 15 of whom die d. Purpura fulminans was seen in 28 patients, of whom 14 (50%) died. A mong the 14 surviving patients who had purpura fulminans, 10 suffered gangrene with deforming autoamputation secondary to the dermal microva scular thrombosis and hemorrhagic necrosis. Evaluation of the induced diffuse intravascular coagulation in 59 patients included studies of t he naturally occurring anticoagulants, focusing on protein C and prote in S. The magnitude of the declining levels of protein C, the degree o f thrombocytopenia, and the presence of fibrin split products are dire ctly related to the clinical severity of the illness (P = .0053). Thus , in individuals with severe disease expression, the risk of purpura f ulminans with death or deformity was significantly increased when the platelet count was <50,000 cells/mm3 (P = .0001) and protein C levels were low (P = .0158). The immaturity of the protein C system in childr en who are <4 years of age may contribute to the rapid and more freque nt pathogenesis of purpura fulminans. Therapy directed at replacement of the naturally occurring anticoagulants, such as protein C, may ulti mately improve the prognosis for individuals with purpura fulminans.