Abstract
Langerhans cell histiocytosis (LCH) is a proliferative disorder of dendritic cells which has evaded efforts to clearly define pathogenesis, diagnostic criteria, and therapeutic response markers. Strides have been made at classification with the recent development of a quantified score of disease severity. Splenic involvement is an indicator of poor prognosis, with spleen size its surrogate marker in evaluation and scoring. We describe a case of pediatric LCH with progressive splenomegaly despite treatment, which when examined at splenectomy revealed no LCH involvement but extramedullary hematopoiesis. These findings thus challenge our understanding of splenomegaly as a marker of disease.
Original language | English (US) |
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Pages (from-to) | 998-999 |
Number of pages | 2 |
Journal | Pediatric Blood and Cancer |
Volume | 58 |
Issue number | 6 |
DOIs | |
State | Published - Jun 2012 |
Keywords
- Extramedullary hematopoiesis
- Langerhans cell histiocytosis
- Risk group
- Splenomegaly