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

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