SHM Converge Scientific Abstract ePoster Gallery-When Ferritin Reaches 81,000: Unravelling Hemophagocytic Lymphohistiocytosis in a 22-Year-Old With Sickle Cell Disease

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This poster describes a 22-year-old man with sickle cell HgSC disease who presented with very high fever, nausea, rising liver enzymes, and later marked hyperferritinemia, ultimately reaching 81,000. His initial evaluation for infection was negative, and imaging did not show pneumonia or acute chest syndrome. Although he was found to be in an acute sickle crisis, his worsening transaminitis, falling hemoglobin and platelets, and persistent systemic inflammation raised concern for hemophagocytic lymphohistiocytosis (HLH), a rare but life-threatening hyperinflammatory syndrome that can mimic sickle cell vaso-occlusive complications.<br /><br />The case highlights the diagnostic challenge of HLH in sickle cell disease because both conditions can present with fever, anemia, pain, organ dysfunction, and elevated ferritin. Standard HLH criteria from 2004 include fever, splenomegaly, cytopenias, hypertriglyceridemia or hypofibrinogenemia, elevated soluble CD25, low NK-cell activity, and hemophagocytosis. In this patient, ferritin, AST/ALT, soluble CD25, anemia, thrombocytopenia, and liver biopsy findings supported HLH. Biopsy showed mononuclear lobular inflammation, Kupffer cell hyperplasia, rare hemophagocytosis, and sickled red blood cells.<br /><br />To manage possible sickle hepatopathy and reduce risk before immunosuppression, the team performed exchange transfusion. When HLH remained likely, corticosteroids were started, after which the patient improved clinically and laboratory values began to downtrend.<br /><br />The authors conclude that HLH should be strongly considered in sickle cell patients with fever, pancytopenia, and multi-organ dysfunction, especially when ferritin is extremely elevated and infection is not found. Prompt recognition and treatment are essential because HLH can be fatal if missed.

Asset Subtitle

Loana M. Sebastian

Meta Tag

Author List Beth Damitz, Loana M. Sebastian

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Sickle Cell Disease

Concept Vaso-occlusive Pain

Concept Fever

Concept Cytopenia

Distinguished Non-Finalist

Presenter Organization Medical College of Wisconsin/Northside Family Medicine Residency

Presenting Author Loana M. Sebastian

Track Adult

Keywords

sickle cell disease

HLH

hyperferritinemia

fever

transaminitis

exchange transfusion

corticosteroids

pancytopenia

liver biopsy

hyperinflammatory syndrome

Hemophagocytic Lymphohistiocytosis

Sickle Cell Disease

Vaso-occlusive Pain

Fever

Cytopenia