SHM Converge Scientific Abstract ePoster Gallery-Hemophagocytic Lymphohistiocytosis Secondary to Sle, Ebv, and HSV

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This case describes a critically ill patient with newly identified severe systemic autoimmune disease consistent with lupus, complicated by multisystem failure and clinically diagnosed hemophagocytic lymphohistiocytosis (HLH).<br /><br />Initial workup showed profound cytopenias and organ involvement: WBC 0.7 x10³/µL, hemoglobin 6 g/dL, platelets 76 x10³/L, elevated AST, and proteinuria. Echocardiogram revealed preserved left ventricular function but a small circumferential pericardial effusion. Autoimmune testing was strongly positive, including ANA, RNP, anti-Smith, SSA, and dsDNA antibodies. Biopsies supported autoimmune organ injury: bone marrow showed megaloblastic changes without malignancy, lymph node biopsy was negative for cancer, and renal biopsy confirmed class IV and V lupus nephritis with moderate activity.<br /><br />The patient then rapidly deteriorated at the time lupus treatment was being initiated, developing hemodynamic instability requiring intubation and severe acidosis requiring hemodialysis. Repeat labs demonstrated marked inflammation and liver injury, including ferritin 40,000, AST 1500 U/L, ALT 1352 U/L, and low fibrinogen. Bronchoscopy/BAL was positive for HSV, and acyclovir was started. Given persistent fevers, extreme hyperferritinemia, liver dysfunction, concurrent infections, and underlying lupus, HLH was diagnosed clinically.<br /><br />Because of impending liver and renal failure, treatment followed an HLH-directed approach with modified dosing: etoposide was reduced to half-dose (75 mg/m²), along with dexamethasone 10 mg/m², rituximab, and acyclovir.<br /><br />The document also emphasizes that HLH is a high-mortality hyperinflammatory syndrome requiring prompt treatment based on clinical suspicion rather than waiting for genetic or immunologic confirmation.

Asset Subtitle

Hinduja Nallamala

Meta Tag

Author List Hinduja Nallamala, Matthew Mitchell, Shelly Kakar

Category Clinical Vignettes

Concept Systemic Lupus Erythematosus

Concept Hemophagocytic Lymphohistiocytosis

Concept Hyperferritinemia

Concept Cytopenia

Concept HLH-94 Protocol

Distinguished Non-Finalist

Presenter Organization Ascension St. Vincent's Hospital

Presenting Author Hinduja Nallamala

Track Adult

Keywords

lupus

hemophagocytic lymphohistiocytosis

HLH

lupus nephritis

hyperferritinemia

cytopenias

autoimmune disease

etoposide

dexamethasone

multisystem failure

Systemic Lupus Erythematosus

Hemophagocytic Lymphohistiocytosis

Hyperferritinemia

Cytopenia

HLH-94 Protocol