Clin Pediatr Hematol Oncol.  2013 Oct;20(2):126-130.

Infant Langerhans Cell Histiocytosis Presented as Isolated Splenomegaly and Prolonged Fever

• Shon HJ
• Jung HJ
• Park JE

Abstract

The isolated spleen involvement is a rare presentation of Langerhans cell histiocytosis (LCH) and can lead to a misdiagnosis. We report a case of LCH in an infant presentated isolated splenomegaly and prolonged fever. A 1-month-old girl admitted due to petechiae, presented with fever for a week and splenomegaly, and showed marginal improvement on empirical treatment. The fever and progressive spleen enlargement persisted over 4 weeks and the cause remained unclear, even after thorough evaluation including bone marrow aspiration and biopsy. LCH was confirmed by splenectomy, and involvement of other organs was excluded. Splenectomy is valuable in the diagnosis of LCH presented with isolated splenomegaly without other organ involvement which may show more aggressive clinical course, but it should be limited to absolutely necessary indications.

Keyword

Fever of unknown origin; Langerhans cell histiocytosis; Splenectomy

MeSH Terms

Biopsy
Bone Marrow
Diagnosis
Diagnostic Errors
Female
Fever of Unknown Origin
Fever*
Histiocytosis, Langerhans-Cell*
Humans
Infant*
Infant, Newborn
Purpura
Spleen
Splenectomy
Splenomegaly*