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Streptococcus pneumoniae-Related Hemophagocytic Lymphohistiocytosis Treated with IVIG and Steroids

Challenging differential diagnosis, Unusual or unexpected effect of treatment, Rare disease

Carissa Y. Dumancas, Hans Alexi Garay Reyes, Juan Cosico, Amrut Savadkar, Soowhan Lah

Department of Medicine, New York Medical College, Metropolitan Hospital Center, New York, NY, USA

Am J Case Rep 2018; 19:25-28

DOI: 10.12659/AJCR.906590

Available online:

Published: 2018-01-08

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.

Keywords: Immunoglobulins, Intravenous, Lymphohistiocytosis, Hemophagocytic, Streptococcus pneumoniae