Haemophagocytic lymphohistiocytosis

Haemophagocytic lymphohistiocytosis (HLH) is a nonmalignant disorder of immune regulation characterised by overproduction of cytokines and diminished immune surveillance. It may affect multiple organs.

It typically affects infants and children 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

Associations

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

Chest

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

Abdomen

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

Brain

Non specific periventricular white-matter abnormalities, brain-volume loss and enlargement of extra-axial fluid spaces may be seen.

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Article information

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

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Cases and figures

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    Case 1: with miliary tuberculosis
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