broaden your horizons (a.k.a. good luck diagnosing this one in the ED): HLH

24 May

HLH (Hemophagocytic LymphoHistiocytosis)

–a condition where there is uncontrolled activation of the cellular immune system. 

–Diagnostic criteria include:

  • idiopathic fever
  • spleenomegaly
  • cytopenias
  • hypertriglyceridemia
  • hypofibrinogenemia
  • lymphadenopathy
  • rash
  • presence of hemophagaocytosis

–Varied skin manifestations:

  • erythroderma
  • generalized purpuric macules and papules
  • morbilliform eruptions. 

–You’re falling asleep right now reading this aren’t you?  

–Basically this is a rare but potentially fatal disease that no Emergency Physician will ever diagnose

–BUT if a patient comes in with this in their medical history, now you (sort of) know what to expect

Submitted by T. Boyd.

Reference(s): Favara BE. Hemophagocytic Lymphohistiocytosis: A He mophagocytic Syndrome. Smnr Diag Path. 1992. Schwartz et al. Lymphohistiocytosis. Emedicine.May 2011, picture


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