Streptococcus pneumoniae-Related Hemophagocytic Lymphohistiocytosis Treated with IVIG and Steroids

Am J Case Rep. 2018 Jan 8:19:25-28. doi: 10.12659/ajcr.906590.

Abstract

BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition that has a poor prognosis due to the ensuing cytokine storm leading to severe organ damage. Current treatment guidelines suggest using a combination of steroid- and etoposide-based chemotherapy. CASE REPORT The authors present a case of a 41-year-old African-American female who presented with symptoms of foodborne illness and who developed multi-organ dysfunction. HLH was suspected because of poor response to broad-spectrum antibiotics with a constellation of findings, including cytopenia, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia. Clinical improvement was noted after administration of intravenous immunoglobulin and dexamethasone while waiting for the soluble interleukin-2 receptor levels; therefore, chemotherapy was not administered. CONCLUSIONS Despite the variable and poor prognosis of HLH, early treatment with steroids and immunosuppressive therapy is crucial to improving the survival rate. The inclusion of immunoglobulin therapy should be considered a treatment option for HLH.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anti-Bacterial Agents / administration & dosage
  • Body Mass Index
  • Dexamethasone / administration & dosage*
  • Diabetes Mellitus, Type 2 / complications
  • Drug Therapy, Combination
  • Female
  • Glucocorticoids / administration & dosage*
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage*
  • Immunologic Factors / administration & dosage*
  • Lymphohistiocytosis, Hemophagocytic / diagnosis
  • Lymphohistiocytosis, Hemophagocytic / microbiology*
  • Lymphohistiocytosis, Hemophagocytic / therapy*
  • Marijuana Use / adverse effects
  • Multiple Organ Failure / drug therapy
  • Multiple Organ Failure / etiology
  • Muscular Diseases / etiology
  • Muscular Diseases / rehabilitation
  • Patient Transfer
  • Prognosis
  • Risk Factors
  • Streptococcus pneumoniae / isolation & purification*
  • Streptococcus pneumoniae / pathogenicity
  • Treatment Outcome

Substances

  • Anti-Bacterial Agents
  • Glucocorticoids
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Dexamethasone