title: Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult.
abstract: Asplenic patients are at increased risk for sepsis and fulminant infection. Sepsis in these patients is typically secondary to encapsulated bacteria, with Streptococcus pneumoniae being the most frequent pathogen. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome). We present the case of a 36-year-old woman, healthy except for splenectomy years prior for idiopathic thrombocytopenic purpura treatment, who presented with fever. Upon presentation to our hospital, three hours after symptoms onset, she had purpura fulminans and shock. Despite timely antimicrobials and maximal resuscitative efforts, her disease progressed and she expired 12 hours after symptoms onset. Autopsy revealed bilateral adrenal hemorrhage; acute adrenal crisis likely contributed to her refractory shock. Prior to her presentation, she had not received guideline-based post-splenectomy care. Sepsis in asplenic patients can be fulminant and rapidly fatal. Streptococcus pneumoniae remains the most frequent cause, despite decreasing rates in recent years related to widespread pneumococcal vaccination. Guideline-based vaccinations and "pill-in-pocket" therapy can be life-saving for asplenic patients. Purpura fulminans represents an extreme manifestation of disseminated intravascular coagulation, is more common in asplenic patients, and portends a poor prognosis. Waterhouse-Friderichsen syndrome can be seen concurrently with purpura fulminans and further portends a poor prognosis; pre-mortem diagnosis requires a high index of suspicion.
authors: Division of Infectious Disease, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215, USA.
authors: Andrew J
authors: AJ
authors: Hale
authors: Division of Infectious Disease, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215, USA.
authors: Mary
authors: M
authors: LaSalvia
authors: Department of Pathology, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215, USA.
authors: James E
authors: JE
authors: Kirby
authors: Department of Medicine, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215, USA.
authors: Allison
authors: A
authors: Kimball
authors: Division of Infectious Disease, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215, USA; Department of Medicine, Alameda Health System, 1411 E 31st St, Oakland, CA 94602, USA.
authors: Rachel
authors: R
authors: Baden
keywords: Asplenia; Purpura fulminans; Sepsis; Streptococcus pneumoniae; Waterhouse-Friderichsen syndrome