Extraosseous langerhans cell histiocytosis

Case contributed by Professor Ali Hekmatnia

Presentation

Anemia with hepatosplenomegaly.

Patient Data

Age: 15 years
Gender: Female

Thin section contrast-enhanced CT scan shows hepatosplenomegaly and linear areas of hypoattenuation along the portal tracts and multiple small hypoattenuated nodules in the liver (represent portal triaditis due to histocytic infiltration) and spleen.

Case Discussion

Bone marrow aspiration of this patient confirmed Langerhans cell histiocytosis (LCH). It is a rare proliferative disorder characterized by granulomatous infiltration of the lung, bone, skin, lymph nodes, liver, spleen, and other organs.

Extraosseous involvement in LCH is much less common than osseous involvement. Liver involvement is relatively common manifestation (40-60%) in multiorgan system disease. Periportal changes are not disease-specific and should be correlated with clinical history and histopathology 1.

Histopathologically, the liver shows periportal langerhans cell infiltration, with features of granulomatous and xanthomatous phases.

Langerhans cell histiocytosis includes four variants:

  • Hashimoto-Pritzker disease
  • Letterer-Siwe disease
  • Hand-Schuller-Christian disease
  • Eosinophilic granuloma
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Case information

rID: 66378
Published: 19th Feb 2019
Last edited: 25th Feb 2019
Inclusion in quiz mode: Included
Institution: Isfahan University of Medical Sciences

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