Case report reveals significant improvement following corticosteroid therapy in HLH with systemic inflammation.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome caused by excessive activation of the immune system. It often mimics severe infections or sepsis and poses a significant diagnostic challenge. We present a rare case of HLH in a 40-year-old female with an unusual presentation involving panniculitis, jaundice, and multi-organ dysfunction. Prompt recognition of persistent cytopenias and systemic inflammation led to the diagnosis through bone marrow biopsy. The patient showed significant improvement following corticosteroid therapy. This case highlights the importance of maintaining a high index of suspicion for HLH in patients with prolonged systemic inflammation and cytopenias. 1. Mellissa R George, Hemophagocytic Lymphohistiocytosis: Review of Etiologies and Management. Journal of Blood Medicine. J Blood Med. 2014 Jun 12:5:69- 86. Doi: 10.2147/JBM.S46255. eCollection 2014. 2. Flavia G N Rosado, MD, Annete S Kim, MD, PPHD. Hemophagocytic Lymphohistiocytosis: An update on Diagnosis and Pathogenesis. American Journal of Clinical Pathology, Volume 139, Issue 6, June 2013 Page 713-727. 3. UpToDate - www.uptodate.com/contents/clinicalfeatures-and-diagnosis-ofhemophagocytic-lymphohistiocytosis/ 4. Robert A Schwartz, MD. Medscape Update Feb 26, 2021.
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