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When to Suspect Hemophagocytic Lymphohistocytosis (HLH, Hemophagocytic Syndrome) as the Cause of a Systemic Inflammatory Response or Sepsis Syndrome

Description

A patient with hemophagocytic lymphohistiocytosis (HLH, hemophagocytic syndrome) may present to the intensive care unit (ICU) with the systemic inflammatory response syndrome, sepsis or septic shock. This is secondary to a massive release of cytokines ("cytokine storm").


 

The patient may present with:

(1) fever

(2) splenomegaly

(3) pancytopenia

(4) multi-organ failure.

 

When to suspect HLH as a possibility in the ICU:

(1) There is a family history of HLH.

(2) The patient was previously healthy with no clear explanation for the current critical illness, or has a history of cancer or autoimmune disease.

(3) The patient is not responding as expected to conventional therapy.

(4) The serum ferritin is very high (> 1,000 ng/mL).

(5) The soluble CD25 (soluble IL-2 receptor) is very high.

 


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