Fatal Cytokine Collision: HLH-AIHA in Advanced AIDS-Case Report and Literature Review

Area of Science:

• Hematology
• Immunology
• Infectious Disease

Background:

• Acquired immunodeficiency syndrome (AIDS) can lead to immune dysregulation and hyperinflammation.
• Hemophagocytic lymphohistiocytosis (HLH) and autoimmune hemolytic anemia (AIHA) are severe hematologic disorders.
• Rarely, HLH and AIHA can co-occur, particularly in the context of advanced AIDS.

Observation:

• A patient with poorly controlled AIDS presented with jaundice, fever, and fatigue, progressing to pancytopenia and hyperbilirubinemia.
• Diagnosis of warm AIHA was confirmed, complicated by HLH criteria fulfillment (high ferritin, sIL-2R).
• Epstein-Barr virus (EBV) and cytomegalovirus (CMV) reactivation were identified.

Findings:

• The patient developed severe complications including ARDS, DVT, and STEMI after HLH treatment with etoposide.
• Etoposide-induced myelotoxicity was catastrophic in this immunocompromised host.
• The patient ultimately succumbed to hemorrhagic shock.

Implications:

• This case emphasizes the diagnostic complexity of HLH-AIHA overlap in AIDS.
• Cytopenias and hyperferritinemia can obscure the underlying cytokine storm.
• There is an urgent need for cytokine-targeted therapies to reduce treatment-related mortality in such patients.