Title: Fulminant Meningococcemia
Authors: Mahmoud Abdel Wahed Omar, Eman Abdou Qurany Kassab
DOI: https://dx.doi.org/10.18535/jmscr/v8i5.43
Abstract
Introduction
Meningococcemia is a life threatening medical emergency that can progress quickly to disseminated intravascular coagulation and multi-organ failure, Meningitis may or may not co-exit. Usually presents with apurpuric rash especially notable on extremities, if adrenal hemorrhage occurs with the rash, the process is called Waterhouse-Frederickson syndrome (Wu, Chen et al. 2020).
Neisseria meningitidis is an aerobic gram-negative coccus that appears typically in pairs (diplococci) with the adjacent sides flattened. The organism is enclosed by a cell envelope containing outer membrane proteins and lipopolysaccharide (endotoxin) and by a polysaccharide capsule. Thirteen serogroups have been identified based on the antigenic structure of the capsular polysaccharide. Six of these serogroups are responsible for most human disease: serogroups A, B, C, Y, W-135, and X (Rosenstein, Perkins et al. 2001).
Disseminated intravascular coagulation (DIC) is of major pathogenic significance in cases of fulminant meningococcemia rapidly leading to death. This syndrome can be detected clinically with a simple and available battery of coagulation tests, prothrombin time, and activated partial thromboplastin time and the platelet count, These tests are sufficiently sensitive in detection of the earliest phases of DIC and providing a rational means for selecting patients requiring anticoagulant therapy (Winkelstein, Songster et al. 1969).
Purpura fulminans is a cutaneous manifestation of disseminated intravascular coagulation, begins with dermal microvessels thrombosis that rapidly leads to hemorrhagic skin necrosis, and gangrene that often necessitates amputation (Agarwal and Sharma 2010).
Sporadic disease occurs more commonly during winter and early spring months and children are predominantly affected. The highest rate of infection is in infants 6 months to 1 year, with a decline in infection rate with age. This explained by passive maternal immunity providing protection in the first 6 months and gradual onset of acquired immunity with age (Rivard, David et al. 1995).
Neisseria meningitidis is the leading cause of infectious Purpura fulminans, followed by infection with other capsulated bacteria such as Haemophilus influenza and Streptococcus pneumoniae (Lécuyer, Borgel et al. 2017).
References
Agarwal, M. P. and V. Sharma (2010). "Purpura fulminans caused by meningococcemia." Cmaj 182(1): E18-E18.
Lécuyer, H., D. Borgel, et al. (2017). "Pathogenesis of meningococcal purpura fulminans." Pathogens and disease 75(3).
Rivard, G. E., M. David, et al. (1995). "Treatment of purpura fulminans in meningococcemia with protein C concentrate." The Journal of pediatrics 126(4): 646-652.
Rosenstein, N. E., B. A. Perkins, et al. (2001). "Meningococcal disease." New England journal of medicine 344(18): 1378-1388.
Winkelstein, A., C. L. Songster, et al. (1969). "Fulminant meningococcemia and disseminated intravascular coagulation." Archives of internal medicine 124(1): 55-59.