Case Challenge #10 presented a 32 year old with SLE who presented with petechiae, pancytopenia, very elevated ferritin, transaminitis, etc.
We asked which of the following would be the most likely diagnosis?
The correct answer is: Hemophagocytic Lymphohistiocytosis (HLH)
- Multisystem disorder of excess immune activation due to either genetic cause (primary) or triggered by secondary causes
- Triggers: Infections (EBV, CMV, HIV, Histo, TB), heme malignancy, autoimmune disease (AOSD, SLE, RA)
- Latter is termed macrophage activation syndrome
- Presentation: Fevers, HSM à MODS, ARDS, shock from cytokine storm.
- Labs findings: Pancytopenia, DIC, abnormal LFTs. Marked elevation in ferritin and triglycerides. Hemophagocytosis on BM or liver biopsy.
- Treatment: Steroids +/- chemo, Rx triggering condition, supportive care
UTSW Internal Medicine