Infant Langerhans Cell Histiocytosis Presented as Isolated Splenomegaly and Prolonged Fever
- Author:
Hyo Jung SHON
1
;
Hyun Joo JUNG
;
Jun Eun PARK
Author Information
1. Department of Pediatrics, Ajou University School of Medicine, Suwon, Korea. free1109@ajou.ac.kr
- Publication Type:Case Report
- Keywords:
Fever of unknown origin;
Langerhans cell histiocytosis;
Splenectomy
- MeSH:
Biopsy;
Bone Marrow;
Diagnosis;
Diagnostic Errors;
Female;
Fever of Unknown Origin;
Fever;
Histiocytosis, Langerhans-Cell;
Humans;
Infant;
Infant, Newborn;
Purpura;
Spleen;
Splenectomy;
Splenomegaly
- From:Clinical Pediatric Hematology-Oncology
2013;20(2):126-130
- CountryRepublic of Korea
- Language:Korean
-
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.
