Haemophagocytic lymphohistiocytosis

Haemophagocytic lymphohistiocytosis (HLH), also known as macrophage activation syndrome, is a nonmalignant disorder of immune regulation characterised by overproduction of cytokines and diminished immune surveillance. It may affect multiple organs and is one of few entities which demonstrate histiocytic emperipolesis on light microscopy.

It typically affects infants and children, as well as adult patients post-transplant 5.

The condition can be primary or secondary. The primary form is thought to have an autosomal recessive inheritance pattern, but with many instances of sporadic occurrence.

The secondary form may occur with several conditions such as 5:

Systemic symptoms can be non-specific but patients can have pancytopenia, coagulopathy, and organomegaly,

Chest radiographs may show alveolar-interstitial opacities with pleural effusions, often with rapid evolution and resolution.

Hepatosplenomegaly, gallbladder wall thickening, hyperechoic kidneys and ascites are common abdominal findings.

Non-specific periventricular white-matter abnormalities, brain-volume loss and enlargement of extra-axial fluid spaces may be seen. Orbital myopathy has also been described.

It is thought to have been first reported by in 1952 by Farquhar and Claireaux who at that time called the disease familial hemophagocytic reticulosis 11.

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Article information

rID: 28189
System: Paediatrics
Tags: stub, cases, cases
Synonyms or Alternate Spellings:
  • HLH
  • Haemophagocytic lymphohistiocytosis (HLH)
  • Hemophagocytic lymphohistiocytosis
  • Hemophagocytic syndrome (HPS)
  • Haemophagocytic syndrome
  • Haemophagocytic syndrome (HPS)
  • Familial hemophagocytic reticulosis

Cases and figures

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    Case 1: with miliary tuberculosis
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